Wild-type (senile) amyloidosis differential diagnosis: Difference between revisions
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[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Wild-type_(senile)_amyloidosis]] | |||
{{ | {{CMG}}{{AE}}{{Sab}}{{HK}} | ||
==Overview== | ==Overview== | ||
Wild-type (senile) amyloidosis can be differentiated from other conditions that present with [[heart failure]], [[polyneuropathy]], and [[organomegaly]]. | |||
==Differentiating Wild-type (Senile) Amyloidosis from other Diseases== | ==Differentiating Wild-type (Senile) Amyloidosis from other Diseases== | ||
==== Differentials Based on Cardiac Involvement (Heart Failure) ==== | |||
Wild-type (senile) amyloidosis should be differentiated from other causes of [[heart failure]]: | |||
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px" align=center | |||
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|+ | |||
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}} | |||
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|History and Symptoms}} | |||
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Physical Examination}} | |||
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Laboratory Findings}} | |||
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Imaging Findings}} | |||
|- | |||
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:Cardiac amyloidosis | |||
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* [[Fatigue]] | |||
* [[Dyspnea]] | |||
* [[Dizziness]] | |||
* [[Orthopnea]] | |||
* [[Peripheral edema]] | |||
* [[Weight loss]] due to cardiac cachexia | |||
* [[Ascites]] | |||
* [[Syncope]] on [[exertion]] | |||
* [[Transthyretin]] (TTR) associated more common in African-Americans during sixth to seventh decade of life | |||
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* Elevated jugular pressure | |||
Periorbital purpura: Often occurs with sneezing, coughing or with minor trauma. Indicates capillary involvement of AL type amyloidosis. | |||
* Macroglossia | |||
* Abnormal phonation | |||
* Hepatomegaly | |||
* Ascites may be present in the setting of heart failure | |||
* Valvular involvement murmurs of mitral and tricuspid regurgitation (systolic). <br /> | |||
<br /> | |||
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* Normocytic mormochromic anemia | |||
* Serum free-light-chain assay positive | |||
* Increased BNP, ANP and β2 microglobulin | |||
* Voltage-to-mass ratio is more sensitive than EKG, 2D Echo and nuclear scanning alone | |||
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* Granular or sparkling appearance of the [[left ventricular]] (LV) [[myocardium]] | |||
* Increased [[Atria|left and right atrial]] volumes and reduced atrial function on [[cardiac MRI]] | |||
* [[Interatrial septum|Atrial septal]] thickening | |||
* Dynamic LV flow | |||
* Mildly or moderately increased wall thickness in the early [[asymptomatic]] phase and severe thickening and [[hypokinesia]] of the [[left ventricular]] posterior wall and [[interventricular septum]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | | |||
:[[Cardiac sarcoidosis]] | |||
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*Asymptomatic conduction abnormalities | |||
*Chest pain | |||
*Congestive heart failure symptoms: | |||
**Fatigue | |||
**Syncope | |||
**Dyspnea | |||
**Chest pain. | |||
**Irregular heartbeats | |||
**Palpitations | |||
**edema<br /> | |||
** | |||
** | |||
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* Ventricular tachyarrhythmia | |||
* Heart block | |||
* Valvular regurgitation | |||
* Pericardial effusion | |||
* Constrictive pericarditis or temponade | |||
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* Serum markers that have been reported as markers of sarcoidosis in general are: | |||
**Serum amyloid A (SAA) | |||
**Soluble interleukin-2 receptor (sIL-2R) | |||
**Lysozyme | |||
**Angiotensin-converting enzyme (ACE) | |||
**Gycoprotein KL-6 | |||
**Hypercalcemia | |||
**Hypercalciuria | |||
**(noncaseating granulomas secrete 1,25 vitamin D) | |||
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*Radionuclide examinations | |||
**Thallium‐201 scintigraphy | |||
**Gallium‐67 scintigraphy | |||
**Positron emission tomography | |||
*Magnetic resonance imaging | |||
*Samples of myocardium with sarcoidosis shows the following: | |||
*Non‐caseating, multinucleated giant cell granuloma in the subendocardium | |||
*Trichrome stain can show a dense band of collagen fibers, encasing aggregate of granulomas and inflammatory cells | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | | |||
:ST Segment Elevation Myocardial Infarction | |||
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*Chest pain with possible radiation to left arm and lower jaw | |||
*Squeezing, crushing chest pain | |||
*Sweating | |||
*Nausea and vomiting | |||
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*Anxious patient in pain with diaphoresis | |||
*Signs of heart failure may be present | |||
*Arrhythmia | |||
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* ST elevation, new left bundle branch block, and Q wave on EKG | |||
* Elevated cardiac biomarkers | |||
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*Either complete or subtotal occlusion of an epicardial coronary artery on coronary angiography | |||
*Confluent hyperenhancement extending from the endocardium | |||
|- | |||
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:Non ST Elevation Myocardial Infarction | |||
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*Crushing, left-sided substernal chest pain or pressure that radiates to the neck or left arm | |||
* | |||
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*Same as ST-elevation MI | |||
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* ST-segment depression or T-wave inversion on EKG | |||
* Elevated cardiac biomarkers | |||
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* | |||
* | |||
|- | |||
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:Pericarditis | |||
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*Chest pain relieved by sitting up and leaning forward and worsened by lying down | |||
*Fever, anxiety, difficulty breathing | |||
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*Pericardial friction rub | |||
*Signs of cardiac tamponade may be present | |||
* | |||
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*PR segment depression and electrical alternans on EKG | |||
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*A flask-shaped, enlarged cardiac silhouette on CXR | |||
*Pericardial thickness of more than 4 mm on MRI | |||
*Pericardial effusion and cardiac chamber indentation or collapse on echo when cardiac tamponade is present | |||
|- | |||
|- | |||
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:Alcoholic Cardiomyopathy | |||
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*History of alcohol abuse | |||
*Fatigue, weakness, anorexia, palpitations, and shortness of breath on activity | |||
*Leg swelling and pedal edema | |||
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*Signs of heart failure such as presence of S3 and S4 heart sounds, pedal edema, and jugular venous distension | |||
*Signs of alcoholic liver disease may be present | |||
* | |||
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*Elevated MCV and MCHC on CBC | |||
*Elevated LDH, AST, ALT, creatine kinase, gammaglutamyl transpeptidase, malic dehydrogenase, and alpha-hydroxybutyric dehydrogenase | |||
*Q waves and non specific ST and T wave changes on EKG | |||
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*Cardiomegaly, pulmonary congestion, and pleural effusions on CXR | |||
*Left ventricular dilatation on echo | |||
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|}<br /> | |||
==== Differentials Based on Presentation As a Multi-Organ System Dysfunction Disorder ==== | |||
<br /> | |||
{| | |||
|- | |||
! style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Organ System Involvement}} | |||
! style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Differential Diagnosis}} | |||
! style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Causes}} | |||
! style="background: #4479BA; width: 300px;" |{{fontcolor|#FFF| Clinical Features}} | |||
! style="background: #4479BA; width: 300px;" |{{fontcolor|#FFF|Laboratory Findings}} | |||
! style="background: #4479BA; width: 300px;" |{{fontcolor|#FFF|Gold Standard Test}} | |||
! style="background: #4479BA; width: 300px;" |{{fontcolor|#FFF|Therapy}} | |||
|- | |||
| rowspan="7" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Polyneuropathy''''' | |||
| | |||
| | |||
| | |||
| | |||
| | |||
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|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[POEMS syndrome]] (Demyelinating)''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Monoclonal]] [[plasma cell]] proliferation | |||
*[[Cytokine]] storm ([[IL-1]], [[Interleukin 6|IL-6]], [[Interleukin 12|IL-12]], [[TNF alpha]], [[VEGF]]) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Symmetrical, ascending chronic progressive [[polyneuropathy]] with both [[Sensory system|sensory]] (pin-prick and vibration) and [[Motor skill|motor]] disability ([[Motor skill|motor]] > [[sensory]]) | |||
* Generalized/extermity [[pain]] | |||
*[[Areflexia]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*Increased number of [[Platelet|thrombocytes]] | |||
*Increased number of [[Red blood cell|erythrocytes]] | |||
*Elevated [[Cerebrospinal fluid|cerebrospinal fluid (CSF)]] [[protein]] content | |||
*Increased number of [[White blood cells|leukocytes]] | |||
*High levels of [[Immunoglobulin G|IgG]] lambda or [[Immunoglobulin A|IgA]] lambda [[M protein|M-protein]] in the [[serum]] | |||
*Increased number of [[Plasma cell|plasma cells]] in the [[bone marrow]] | |||
*Increased serum [[Vascular endothelial growth factor|VEGF]] level | |||
*Elevated levels of antitiroglobulin [[antibody]] and antithyroid peroxydase [[antibody]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[POEMS syndrome diagnostic criteria|International Myeloma Working Group (IMWG) clinical and laboratory diagnostic criteria]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Metabolic syndrome|Metabolic Syndrome]] (Axonal pathology)''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Diabetes mellitus]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Symmetric sensorimotor distal polyneuropathy | |||
* Asymmetric proximal neuropathy | |||
*[[Oculomotor nerve palsy|3rd nerve palsy]] | |||
*[[Carpal tunnel syndrome|Carpel tunnel syndrome]] | |||
*[[Autonomic neuropathy]] | |||
* "Glove and stocking" type pain | |||
*[[Muscle wasting]] | |||
*[[Hammer toe|Hammer toes]] | |||
*[[Polyuria]] | |||
*[[Polydipsia]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Uncontrolled [[hyperglycemia]] | |||
* Slowed [[Nerve conduction study|nerve conduction]] | |||
*[[Small fiber peripheral neuropathy|Small fiber dysfunction]] | |||
*[[Monofilament|Monofilament testing]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Fasting blood sugar|Fasting blood sugar level]] greater than equal to 126 mg/dl on 2 separate occasions | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Diabetes mellitus medical therapy|Anti-diabetic therapy]] | |||
*[[Gabapentin]] | |||
*[[Carbamazepine]] | |||
*[[Foot care]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Vitamin deficiencies|Vitamin Deficiencies]] (Axonal Pathology)''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Vitamin B12 deficiency]] (Decreased [[S-Adenosyl methionine|S-adenosyl methionine]]) | |||
*[[Thiamine deficiency|Vitamin B1 deficiency]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Primarily [[sensory]] deficits | |||
* Vibration and [[proprioception]] affected | |||
*[[Gait abnormality|Gait abnormalities]] | |||
*[[Cognitive impairment]] | |||
*[[Irritability]] | |||
*[[Glossitis]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Anemia]] ([[Megaloblastic Anemias|megaloblastic]] in case of [[Vitamin B12 deficiency|B12 deficiency]]) | |||
* Decreased [[serum]] [[Vitamin B12]] levels (< 200 pg/ml) | |||
*[[Methylmalonic acidemia|Elevated methylmalonic acid]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Serum]] [[Vitamin B12]] levels | |||
*[[Methylmalonic acid|Methylmalonic acid levels]] | |||
*[[Intrinsic factor|Intrinsic factor antibodies]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Vitamin B12]] supplement ([[parenteral]]) | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Guillain-Barré syndrome|Guillain-Barre Syndrome]] (Demyelinating)''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Anti-ganglioside antibodies|Anti-ganglioside]] and anti-[[myelin]] antibodies | |||
*[[Viral]] infections: | |||
**[[Epstein Barr virus]] | |||
**[[Human Immunodeficiency Virus (HIV)|HIV]] | |||
**[[Cytomegalovirus]] | |||
**[[Varicella Zoster Virus|Varicella Zoster virus]] | |||
*[[Bacterial]] infections: | |||
**[[Campylobacter]] infection | |||
**[[Mycoplasma pneumoniae]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Rapid onset and quick progression | |||
* Progression stops after 2-3 weeks | |||
* Bilateral ascending [[Paraesthesia|paraesthesias]] and [[paralysis]] (generalized) | |||
*[[Muscle weakness|Weakness]] | |||
*[[Ataxia]] | |||
*[[Areflexia]] | |||
* No fever | |||
* 4 sub-types: | |||
**[[Acute inflammatory demyelinating polyneuropathy]] | |||
**[[Acute motor axonal neuropathy]] | |||
**Acute motor and sensory axonal neuropathy | |||
**[[Miller Fisher syndrome]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Delayed F waves | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Clinical diagnostic criteria (progressive weakness of more than two [[limbs]], [[areflexia]], and progression for no more than four weeks) | |||
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*[[Intravenous]] [[immunoglobulins]] | |||
*[[Plasma]] exchange | |||
*[[Mechanical ventilation|Respiratory support]] | |||
*[[DVT]]/[[PE]] [[prevention]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic inflammatory demyelinating polyneuropathy|Chronic Inflammatory Demyelinating Polyneuropathy]] (CIDP) (Mixed axonal and demyelinatiing)''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Abnormal [[immune response]] (both [[Immunoglobulin G|IgG]] based [[Humoral immunity|humoral]] and [[Cell mediated immunity|T-Cell mediated]]) response to unknown [[antigen]] (possible culprits include [[myelin]] [[proteins]] P0, P2 and PMP22) | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Slow onset and gradual progression | |||
*[[Relapse|Relapsing]] and remitting course | |||
* Symmetrical [[Proximal muscle weakness|proximal]] and [[Distal muscle weakness|distal motor]] and [[Sensory system|sensory weakness]] (legs>arms) | |||
*[[Foot drop]] | |||
*[[Numbness]], [[tingling]] and [[pain]] | |||
*[[Areflexia]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Elevated [[CSF]] [[protein]] ([[oligoclonal bands]] with normal [[White blood cells|WBCs]]) | |||
* Slowed [[Nerve conduction study|motor nerve conduction velocities]] | |||
* Prolonged distal [[Motor skill|motor]] latencies (period between F wave and initial stimulation) | |||
* Delayed F wave latencies (recorded from the [[feet]], hence called "F" waves) | |||
*[[MRI]] contrast enhancement and enlargement of [[Vertebra|T2]] [[spinal segments]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* EFNS/PNS criteria | |||
* Koski criteria | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Corticosteroids]] | |||
*[[Intravenous immunoglobulin]] ([[IVIG]]) | |||
*[[Immunosupressive drug|Immunosupressants]] ([[Alemtuzumab|Alemtuzemab]] [[Azathioprine]] [[Cyclophosphamide]] [[Cyclosporine|Cyclosporin]] [[Etanercept]] [[Interferon-alpha]]) | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multifocal motor neuropathy|Multifocal Motor Neuropathy]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Immune response|Abnormal immune response]] ([[Anti-ganglioside antibodies|Anti ganglioside]] [[Anti-ganglioside antibodies|GM-1]] [[IgM]] [[antibodies]]) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Progressive, asymmetric, distal and upper [[Limb (anatomy)|limb]] predominant weakness | |||
* No significant [[sensory]] abnormalities | |||
*[[Areflexia]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Elevated [[CSF]] [[protein]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Clinical criteria (EFNS/PNS): | |||
** Slowly progressive or step-wise progressive, focal, asymmetric [[limb]] weakness; i.e., [[Motor skill|motor]] involvement in the [[motor nerve]] distribution of at least two nerves for > 1 month. | |||
** No objective [[Sensory system|sensory]] abnormalities except for minor vibration sense abnormalities in the [[lower limbs]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Intravenous immunoglobulin|Intravenous immunoglobulins]] | |||
*[[Cyclophosphamide]] | |||
*[[Rituximab]] | |||
|- | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Organ System Involvement'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Differential Diagnosis'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Causes'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Features'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Laboratory Findings}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Gold Standard Test}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Therapy'''}} | |||
|- | |||
| rowspan="7" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Organomegaly (Hepatosplenomegaly and Lymphadenopathy)''''' | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Malaria]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Plasmodium falciparum]] | |||
*[[Plasmodium ovale|P. ovale]] | |||
*[[P. malariae]] | |||
*[[Plasmodium knowlesi|P. knowlesi]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Tertian ([[Plasmodium vivax|vivax]], [[Plasmodium ovale|ovale]], [[Plasmodium falciparum|falciparum]]), quartan (malariae), quotidian fever ([[Plasmodium knowlesi|knowlesi]]) | |||
*[[Vector]] is female [[Anopheles]] mosquito | |||
*[[Hepatosplenomegaly]] | |||
*[[Lymphadenopathy]] | |||
*[[Jaundice]] | |||
*[[Icterus (medicine)|Icterus]] | |||
*[[Tachycardia]] | |||
*[[Tachypnea]] | |||
*[[Productive cough]] | |||
*[[Hematuria]] | |||
*[[Altered mental status]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Microcytic anemia]] | |||
*[[Malaria differential diagnosis|Thick and thin blood films]] ([[Giemsa stain|Giemsa staining]]) | |||
* Rapid diagnostic test ([[antigen]] detection | |||
*[[Polymerase chain reaction]] ([[Polymerase chain reaction|PCR]]) | |||
*[[Enzyme linked immunosorbent assay (ELISA)|Enzyme linked immunosorbent assay]] ([[Enzyme linked immunosorbent assay (ELISA)|ELISA]]) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Malaria differential diagnosis|Thick and thin films]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Non-[[Plasmodium falciparum|falciparum]] species: | |||
**[[Chloroquine]] (in susceptible) | |||
**[[Artemisinin]] plus [[Mefloquine|mefloquin]] or [[lumefantrine]] (in [[chloroquine]] resistant) | |||
*[[Plasmodium falciparum|Falciparum]] species: | |||
**[[Chloroquine]] (in susceptible) | |||
**[[Artemether]] plus [[lumefantrine]] (in [[Chloroquine|chloroquin]] resistant) OR | |||
**[[Artesunate]] plus [[Mefloquine|mefloquin]] OR | |||
**[[Artesunate]] plus [[Sulfadoxine|sulfadoxine-pyrimethamine]] | |||
**[[Atovaquone-Proguanil|Atovaquone plus proguanil]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Kala-azar]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Leishmaniasis|Leshmania donovani]] | |||
* L. infantum | |||
* L. chagasi | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Fever]] | |||
* Vector is [[sandfly]] | |||
*[[Hepatosplenomegaly]] | |||
*[[Lymphadenopathy]] | |||
*[[Hyperpigmentation]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Anemia]] | |||
*[[Direct agglutination test]] (DAT) | |||
* rk39 dipstick | |||
*[[Enzyme linked immunosorbent assay (ELISA)|ELISA]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Splenic]] aspiration | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Liposomal amphotericin B]] | |||
*[[Sodium stibogluconate]] | |||
*[[Pentamidine]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Hepatitis|Infective Hepatitis]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Hepatitis A virus|Hepatitis A virus (HAV)]] | |||
*[[HBV]] | |||
*[[Hepatitis C|HCV]] | |||
*[[Hepatitis D|HDV]] (co-infection with [[HBV]]) | |||
*[[Hepatitis E|HEV]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Fever]] | |||
* Transmitted via [[fecal-oral route]] ([[Hepatitis A|HAV]], [[Hepatitis B virus|HBV]], [[HDV]], [[HEV]]), infected sera ([[HCV]]), [[sexual contact]] with infected individuals | |||
*[[Hepatosplenomegaly]] (may become shrunken in cases of [[cirrhosis]] due to chronic infection) | |||
*[[Lymphadenopathy]] | |||
*[[Jaundice]] | |||
*[[Palmar erythema]] | |||
*[[Spider angiomata]] | |||
*[[Gynecomastia]] | |||
*[[Arthritis-dermatitis syndrome]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Antigen]] and [[antibody]] detection | |||
* Total and direct [[bilirubin]] (increased) | |||
* Severe disease is often associated with persistent [[bilirubin]] levels >340 mmol/L | |||
*[[ALT]] and [[AST]] (increased) | |||
*[[Alkaline phosphatase]] (normal or mildly elevated) | |||
*[[Prothrombin time]] (prolonged from synthetic defect, caused by hepatocellular [[necrosis]]) | |||
*[[Total protein]] (decreased) | |||
*[[Globulin]] (mildly elevated) | |||
* Initial [[lymphopenia]] and [[neutropenia]], followed by relative [[lymphocytosis]] | |||
*[[Anemia|Low hemoglobin]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Antigen]] and [[antibody]] detection | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Interferon]] ([[IFN]]) | |||
*[[Antivirals|Nucleoside analogs]] | |||
*[[Antivirals|Nucleotide analogs]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic myelogenous leukemia|Chronic Myelogenous Leukemia]] (CML)''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Philadelphia chromosome|BCR/ABL gene]] fusion product due to [[Chromosomal translocation|translocation]] [[mutation]] [[Philadelphia chromosome|t(9;22)]](q34;q11) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Fever]] | |||
*[[Weight loss]] | |||
*[[Hepatosplenomegaly]] | |||
*[[Lymphadenopathy]] | |||
*[[Bruises]] | |||
*[[Petechiae]] | |||
*[[Ulcers]] | |||
*[[Vesicles]] | |||
*[[Malaise]] | |||
*[[Early satiety]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Anemia]] | |||
*[[Leukocytosis]] (median of 100,000/µL) with a [[left shift]] | |||
*[[Thrombocytosis]] | |||
*[[Blast|Blasts]] usually <2% | |||
* Absolute [[basophilia]] | |||
* Absolute [[eosinophilia]] | |||
*[[Monocytosis]] | |||
*[[Thrombocytosis]] | |||
*[[Thrombocytopenia]] suggests an alternative diagnosis or the presence of advanced stage | |||
* Elevated [[uric acid]] | |||
* Elevated [[histamine]] levels | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Fluorescence in situ hybridization|Fluoroscent insitu hybridization (FISH)]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Imatinib]] | |||
*[[Dasatinib]] | |||
*[[Nilotinib]] | |||
*[[Bosutinib]] | |||
*[[Ponatinib]] | |||
*[[Cytarabine]] | |||
*[[Cytarabine|HDAC]] (high-dose [[cytarabine]]) | |||
*[[Hydroxyurea]] | |||
*[[Busulfan]] | |||
*[[Busulfex]] | |||
*[[Stem cell transplantation]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Lymphoma]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Various causes based on type: | |||
**[[Hodgkin's lymphoma|Hodgkin's]] | |||
**[[Non-Hodgkin lymphoma|Non-Hodgkin's]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Fever]] | |||
*[[Weight loss]] | |||
*[[Lymphadenopathy]] | |||
*[[Hepatosplenomegaly]] | |||
*[[Night sweats]], constant [[fatigue]] | |||
* Purplish scaly rash in cases of [[cutaneous lymphoma]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Erythrocyte sedimentation rate|Elevated ESR]] | |||
*[[C-reactive protein|Increased CRP]] | |||
*[[Lactate dehydrogenase|Increased LDH]] | |||
*[[Anemia of chronic disease]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Lymph node biopsy]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Primary amyloidosis|Primary (AL) Amyloidosis]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Aggregation and deposition of [[immunoglobulin light chains]] that are usually produced by [[plasma cell]] clones | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Nephrotic syndrome]] ([[peripheral edema]]) | |||
*[[Restrictive cardiomyopathy]] ([[fatigue]], [[dyspnea]], [[syncope]]) | |||
*[[Peripheral neuropathy]] ([[numbness]], [[tingling]]) | |||
*[[Hepatomegaly]] with elevated [[liver enzymes]] | |||
*[[Macroglossia]] | |||
*[[Purpura]] | |||
*[[Bleeding diathesis]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Typical green birefringence under polarized light after Congo red staining (appears in red under normal light) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Congo red staining | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Melphalan-prednisone/dexamethasone | |||
* Dexamethasone plus Cyclophosphamide-thalidomide | |||
* Stem cell transplantation | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Gaucher's disease|Gaucher's Disease]]''' | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[GBA (gene)|GBA gene]] [[mutation]] | |||
* Aberrant metabolism of [[glucocerebroside]] ([[lipid]]) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Hydrops fetalis]] | |||
* Dry, scaly skin ([[ichthyosis]]) or other [[skin]] abnormalities | |||
*[[Hepatosplenomegaly]] | |||
* Distinctive facial features | |||
*[[Neurological disorder|Neurological problems]] | |||
*[[Gall stones]] | |||
*[[Growth retardation]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Hypocholesterolemia]] | |||
*[[Splenic]] nodules | |||
*[[Cytopenias]] (especially [[thrombocytopenia]]) | |||
* Increased [[ferritin]] levels | |||
* Increased tartarate resistant acid phosphatase (TRAP) levels | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Enzyme assay for [[glucocerebrosidase]] | |||
*[[DNA|DNA analysis]] for [[GBA (gene)|GBA mutation]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Enzyme replacement therapy|Enzyme replacement]] | |||
*[[Splenectomy]] | |||
*[[Blood transfusion]] | |||
|- | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF| |'''Organ System Involvement'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Differential Diagnosis'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Causes'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Features'''}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Laboratory Findings}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|Gold Standard Test}} | |||
| style="background: #4479BA; width: 200px;" |{{fontcolor|#FFF|'''Therapy'''}} | |||
|- | |||
| rowspan="6" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Cardiac Failure''''' | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Cardiac amyloidosis (AL and ATTRwt) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Monoclonal plasma cell proliferation | |||
* Extracellular amyloid fibril deposition <br /> | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Fatigue | |||
* Dyspnea | |||
* Dizziness | |||
* Orthopnea | |||
* Peripheral edema | |||
* Weight loss due to cardiac cachexia | |||
* Ascites | |||
* Syncope on exertion | |||
* Transthyretin (ATTRwt) associated more common in African-Americans during sixth to seventh decade of life | |||
** | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Normocytic mormochromic anemia | |||
* Serum free-light-chain assay positive | |||
* Increased BNP, ANP and β2 microglobulin | |||
* Voltage-to-mass ratio is more sensitive than EKG, 2D Echo and nuclear scanning alone <br /> | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Biopsy: | |||
* Diffuse deposition of amorphous hyaline material (nodular pattern - 8 to15 nm in diameter), in mesangium (weakly staining with periodic acid-Schiff (PAS) | |||
<br /> | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Supportive care | |||
* Tafamidis | |||
* Melphalan-prednisone/dexamethasone | |||
* Dexamethasone plus Cyclophosphamide-thalidomide | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Cardiac sarcoidosis]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* The causes are not fully known. | |||
* Over-reaction of the immune system after exposure to an infectious agent (bacteria or viruses), chemical, or allergen. | |||
*Excessive inflammation and the clustering of white blood cells. | |||
** | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Asymptomatic conduction abnormalities | |||
* Chest pain | |||
* Congestive heart failure symptoms: | |||
** Fatigue | |||
** Syncope | |||
** Dyspnea | |||
** Chest pain. | |||
** Irregular heartbeats | |||
** Palpitations | |||
** edema | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
Serum markers that have been reported as markers of sarcoidosis in general are: | |||
* Serum amyloid A (SAA) | |||
* Soluble interleukin-2 receptor (sIL-2R) | |||
* Lysozyme | |||
* Angiotensin-converting enzyme (ACE) | |||
* Gycoprotein KL-6 | |||
* Hypercalcemia | |||
* Hypercalciuria | |||
* (noncaseating granulomas secrete 1,25 vitamin D) | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Biopsy: samples of myocardium with sarcoidosis shows the following: | |||
* Non‐caseating, multinucleated giant cell granuloma in the subendocardium | |||
* Trichrome stain can show a dense band of collagen fibers, encasing aggregate of granulomas and inflammatory cells | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Corticosteroid treatment | |||
* Antiarrhythmic treatment | |||
* Pacemakers and defibrillators | |||
* Cardiac transplantation | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Hypertrophic obstructive cardiomyopathy]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*[[Mutations|Mutation]] in sarcomeric protein (beta myosin heavy chain and myosin binding protein C) | |||
*[[Autosomal dominant]] inheritance | |||
* | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" |<br /> | |||
* [[Chest pain]] (also known as [[Angina pectoris|angina]]) | |||
* [[Dizziness]] | |||
* [[Dyspnea]] ([[shortness of breath]]) which is due to increased stiffness of the [[hypertrophied left ventricle]] | |||
* [[Exercise intolerance]] | |||
* [[Fainting]], [[presyncope]] or frank [[syncope]], especially during exercise | |||
* [[Fatigue]]) | |||
* [[Light-headedness]] | |||
* [[Shortness of breath]] | |||
* [[Reduced activity tolerance]] | |||
* [[Shortness of breath]] | |||
* [[Sudden cardiac death]] | |||
<br /> | |||
** | |||
** | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Increased [[Brain natriuretic peptide|BNP]] | |||
* Increased [[creatine kinase]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Echocardiography: | |||
** [[Left ventricular]] asymmetric hypertrophy | |||
** Parasternal long axis shows relationship of the septal hypertrophy and the outflow tract | |||
** Left ventricular diastolic dysfunction | |||
** SAM (systolic anterior motion) of the mitral leaflet | |||
** Mid-systolic closure of the aortic valve | |||
** Late peaking, high velocity flow in the outflow tract | |||
** Variability of obstruction with maneuvers (exercise, amyl nitrate inhalation, and post-PVC beats) <br /> | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Beta blockers]] | |||
* [[Calcium channel blocker|Calcium channel blockers]] | |||
* [[Septal myectomy]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Alcoholic cardiomyopathy]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Alcohol consumption | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Exercise intolerance]] | |||
*[[Fainting]], [[presyncope]] or frank [[syncope]], especially during exercise | |||
*[[Fatigue]]) | |||
*[[Light-headedness]] | |||
*[[Shortness of breath]] | |||
*[[Reduced activity tolerance]] | |||
*[[Shortness of breath]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" |<br /> | |||
* Elevated [[mean corpuscular volume]] ([[MCV]]) and [[mean corpuscular hemoglobin]] (MCHC) | |||
* Mild [[thrombocytopenia]] | |||
* Elevated [[LDH]], [[AST]], [[ALT]], [[creatine kinase]], malic dehydrogenase and alpha-hydroxybutyric dehydrogenase | |||
* Elevated gammaglutamyl transpeptidase | |||
* Serum concentrations of [[magnesium]] and [[zinc]] may be reduced | |||
<br /> | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Endomyocardial biopsy]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Restriction of dietary salt | |||
* [[ACE inhibitor|ACE inhibitors]] or [[angiotensin II receptor blockers]] | |||
* [[Beta blockers]] | |||
* [[Diuretics]] | |||
* [[Digoxin]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[ST elevation myocardial infarction|ST-elevation myocardial infarction]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Myocardial ischemia]] | |||
* [[Atherosclerosis]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Exercise intolerance]] | |||
*[[Fainting]], [[presyncope]] or frank [[syncope]], especially during exercise | |||
*[[Fatigue]]) | |||
*[[Light-headedness]] | |||
*[[Shortness of breath]] | |||
*[[Reduced activity tolerance]] | |||
*[[Shortness of breath]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Increased [[Cardiac troponin|cardiac troponins]] | |||
* Increased [[Lactate dehydrogenase|LDH]] | |||
* [[Leukocytosis]] | |||
* [[ST segment elevation]] on EKG | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Elevation of [[Cardiac troponin|cardiac troponins]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Percutaneous coronary intervention or coronary artery bypass graft | |||
* [[Aspirin]] | |||
* [[Clopidogrel]] | |||
* [[Beta blockers]] | |||
* [[Diuretics]] | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Pericarditis]] | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[HIV]] | |||
* [[Dressler's syndrome]] | |||
* [[Tuberculosis]] | |||
* [[Uremia]] | |||
* [[Radiation]] | |||
* [[Malignancy]] | |||
* | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Chest pain]] (relieved by sitting up and leaning forward and is worsened by lying down) | |||
* [[Cough]] (either dry or productive) | |||
* [[Fever]] | |||
* [[Fatigue]] | |||
* [[Anxiety]] | |||
* [[Breathlessness]] | |||
<br /> | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* [[Creatine kinase]]: [[Acute pericarditis]] may be associated with a modest increase in serum [[creatine kinase]]-MB (CK-MB) depending upon the extent of involvement of the underlying [[myocardium]]. | |||
* Increased [[Cardiac troponin I (cTnI) and T (cTnT)|cardiac troponin-I (cTnI)]] | |||
* Increased [[LDH]] | |||
* Increased serum [[myoglobin]] | |||
* Increased [[SGOT]] ([[AST]]) | |||
<br /> | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
* Two of the following four criteria: | |||
** Pericarditic chest pain | |||
** [[Pericardial rubs|Pericardial rub]] | |||
** New widespread [[ST-segment elevation]] or [[PR depression]] | |||
** New or worsening [[pericardial effusion]]. | |||
* Supporting findings can include elevation of inflammatory markers ([[C-reactive protein (CRP)|C-reactive protein]], [[Erythrocyte sedimentation rate|ESR]], [[white blood cell count]]), and evidence of pericardial inflammation on imaging([[Computed tomography|CT scan]] and [[cardiac MRI]]). | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
|} | |||
==References== | ==References== |
Latest revision as of 15:38, 24 December 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]Syed Hassan A. Kazmi BSc, MD [3]
Overview
Wild-type (senile) amyloidosis can be differentiated from other conditions that present with heart failure, polyneuropathy, and organomegaly.
Differentiating Wild-type (Senile) Amyloidosis from other Diseases
Differentials Based on Cardiac Involvement (Heart Failure)
Wild-type (senile) amyloidosis should be differentiated from other causes of heart failure:
Differential Diagnosis | History and Symptoms | Physical Examination | Laboratory Findings | Imaging Findings |
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Periorbital purpura: Often occurs with sneezing, coughing or with minor trauma. Indicates capillary involvement of AL type amyloidosis.
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Differentials Based on Presentation As a Multi-Organ System Dysfunction Disorder
Organ System Involvement | Differential Diagnosis | Causes | Clinical Features | Laboratory Findings | Gold Standard Test | Therapy |
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Polyneuropathy | ||||||
POEMS syndrome (Demyelinating) |
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Metabolic Syndrome (Axonal pathology) |
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Vitamin Deficiencies (Axonal Pathology) |
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Guillain-Barre Syndrome (Demyelinating) |
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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing) |
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Multifocal Motor Neuropathy |
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Organ System Involvement | Differential Diagnosis | Causes | Features | Laboratory Findings | Gold Standard Test | Therapy |
Organomegaly (Hepatosplenomegaly and Lymphadenopathy) | Malaria |
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Kala-azar |
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Infective Hepatitis |
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Chronic Myelogenous Leukemia (CML) |
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Lymphoma |
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Primary (AL) Amyloidosis |
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Gaucher's Disease |
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Organ System Involvement | Differential Diagnosis | Causes | Features | Laboratory Findings | Gold Standard Test | Therapy |
Cardiac Failure | Cardiac amyloidosis (AL and ATTRwt) |
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Cardiac sarcoidosis |
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Serum markers that have been reported as markers of sarcoidosis in general are:
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Hypertrophic obstructive cardiomyopathy |
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Alcoholic cardiomyopathy |
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ST-elevation myocardial infarction |
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Pericarditis |
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