Differentiating Antiphospholipid syndrome from other diseases: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Antiphospholipid_syndrome]] | |||
{{CMG}}{{AE}}{{FT}} | {{CMG}}{{AE}}{{FT}} | ||
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[[Pulmonary embolism]], [[Budd-Chiari syndrome]] | [[Pulmonary embolism]], [[Budd-Chiari syndrome]] | ||
! | ![[Doppler ultrasound|Doppler]] ultrasonography for recurrent [[Deep vein thrombosis|DVT]] | ||
!Noninflammatory bland [[thrombosis]] without perivascular [[inflammation]] | !Noninflammatory bland [[thrombosis]] without perivascular [[inflammation]] | ||
!Hx of [[thrombosis]] and [[antiphospholipid antibodies]] | !Hx of [[thrombosis]] and [[antiphospholipid antibodies]] | ||
Line 75: | Line 75: | ||
! | ! | ||
! | ! | ||
! | !+ | ||
! | ! [[Leukopenia]], [[Lymphopenia]], [[Anemia]], [[Thrombocytopenia]] | ||
! | !↑ | ||
! | ![[Anti-dsDNA antibody|Anti dsDNA]], [[ANA]] | ||
! | !↑[[Creatinine|Cr]] or [[Blood urea nitrogen|BUN]], | ||
! | |||
! | ↑[[ALT]] or [[AST]], [[Proteinuria]] | ||
! | !Interstitial lung disease, [[Pneumonitis]], [[Pulmonary emboli]], [[Hemorrhage|Alveolar hemorrhage]] | ||
! | ![[Central nervous system|Central nervous system (CNS)]] [[Systemic lupus erythematosus|lupus]]<nowiki/>white-matter changes in [[MRI]] | ||
! | !Staging [[lupus nephritis]] | ||
![[Anti-dsDNA antibody|Anti-dsDNA antibody test]] | |||
![[Skin rashes]] or [[photosensitivity]] | |||
|- | |- | ||
! rowspan="7" |[[Thrombosis]] | ! rowspan="7" |[[Thrombosis]] | ||
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![[Idiopathic thrombocytopenic purpura|ITP]] | ![[Idiopathic thrombocytopenic purpura|ITP]] | ||
! | ! | ||
! | !+/- | ||
! | !- | ||
! | !+ | ||
! | ![[Petechia|Petechiae]] | ||
! | ![[Splenomegaly]] | ||
! | !+ | ||
! | ![[Anemia]], [[Thrombocytopenia]] | ||
! | !- | ||
! | !Anti platelet factor 4 antibodies | ||
! | ![[HIV]], [[ANA]] | ||
! | !R/O other causes | ||
! | !- | ||
! | !Increased number of normal morphologic [[megakaryocytes]] | ||
! | !Clinical findings coupled with [[thrombocytopenia]] | ||
! | ![[Easy bruising]], [[Purpura]] | ||
|- | |- | ||
![[HELLP syndrome|HELLP]] | ![[HELLP syndrome|HELLP]] | ||
!- | |||
!- | |||
!- | |||
!- | |||
! | ! | ||
! | ![[Hepatomegaly]] | ||
! | !+ | ||
! | ![[Anemia]], [[Thrombocytopenia]], [[Schistocytes]] | ||
! | !- | ||
! | !- | ||
! | !↑[[Bilirubin]], ↓[[Haptoglobin]], ↑[[Lactate dehydrogenase|LDH]], ↑[[Creatinine|Cr]] | ||
! | !Cortical hypodense areas in the [[Occipital lobe|occipital lobes]], Diffuse [[cerebral edema]] | ||
! | !Ultrasonography shows [[Fetal growth retardation|Poor fetal growth]], [[Oligohydramnios]], Abnormal [[umbilical artery]] | ||
! | !- | ||
! | ![[Urinalysis|24-hour urine study]] | ||
! | ![[Seizure]], [[Edema]] | ||
|- | |- | ||
! colspan="2" |[[Paroxysmal nocturnal hemoglobinuria|Paroxysmal nocturnal hemoglobinuria (PNH)]] | ! colspan="2" |[[Paroxysmal nocturnal hemoglobinuria|Paroxysmal nocturnal hemoglobinuria (PNH)]] | ||
!+ | |||
!+ | |||
!- | |||
!+/- | |||
! | ! | ||
! | ! | ||
! | ! | ||
! | ![[Anemia]], [[Reticulocyte|reticulocytosis]], | ||
Increased [[lactate]] [[dehydrogenase]] (LDH) and [[bilirubin]] | |||
!- | |||
!Direct [[Coombs test|antiglobulin]] (Coombs) testing (DAT) | |||
! | !Increased [[haptoglobin]] | ||
! | !- | ||
! | !- | ||
! | !Bone marrow biopsy shows normocellular or hypercellular bone marrow with [[Red blood cell|erythroid]] [[hyperplasia]] | ||
! | ![[Flow cytometry]] | ||
! | |||
! | |||
! | ! | ||
|- | |- | ||
! colspan="2" |[[Heparin-induced thrombocytopenia|Heparin-induced thrombocytopenia (HIT)]] | ! colspan="2" |[[Heparin-induced thrombocytopenia|Heparin-induced thrombocytopenia (HIT)]] | ||
!+ | |||
!+ | |||
!- | |||
!- | |||
! | ! | ||
* Redness of skin | |||
* [[Purpura]] | |||
* Bleeding from the skin | |||
!- | |||
!+ | |||
![[Thrombocytopenia]] | |||
!+ | |||
!Anti-heparin-PF4 IgG | |||
! | ! | ||
* Serotonin release assay (SRA) | |||
* Heparin induce platelet aggregation (HIPA) assays | |||
* Solid-phase immunoassay (SPI) (H-PF4 enzyme-linked immunosorbent assay [[[Enzyme linked immunosorbent assay (ELISA)|ELISA]]]) | |||
* Particle gel immunoassay (PIFA) | |||
![[Stroke]], [[pulmonary embolism]], [[mesenteric ischemia]], intraabdominal or [[Retroperitoneum|retroperitoneal]] bleeding, or acute limb [[ischemia]]. | |||
!- | |||
![[Schistocytes]] | |||
! | !Serotonin release assay | ||
! | !Limb gangrene | ||
! | |||
! | Skin necrosis | ||
! | |||
|- | |- | ||
! colspan="2" |[[Myeloproliferative disease|Myeloproliferative diseases]] | ! colspan="2" |[[Myeloproliferative disease|Myeloproliferative diseases]] | ||
!+ | |||
!+ | |||
!+ | |||
!+ | |||
! | ! | ||
* [[Petechiae]] | |||
* Anal pruritis | |||
![[Splenomegaly]] | |||
!- | |||
!↑[[White blood cells|WBCs]], predominantly [[Neutrophil|neutrophils]] | |||
!+ | |||
!- | |||
! | ! | ||
* [[Bone marrow aspiration]] | |||
* [[Biopsy|Trephine biopsy]] | |||
* [[Lumbar puncture]] | |||
* [[Lymph node biopsy]]. | |||
! | ! | ||
* Enlarged lymph nodes | |||
* [[Splenomegaly]] | |||
* Splanchnic venous thrombosis | |||
! [[PET]] scan- [[metastasis]] in bone marrow | |||
! Terminal [[myeloid]] cell expansion | |||
![[Philedelphia chromosome]] by PCR or BCR/ABL by FISH | |||
! | ! | ||
* [[Bruising]] | |||
* [[Epistaxis]] | |||
* [[Bleeding gums]] | |||
* [[Menorrhagia]] | |||
* [[Metrorrhagia]] | |||
* [[Petechiae]] | |||
|- | |- | ||
! rowspan="3" |[[Recurrent pregnancy loss]] | ! rowspan="3" |[[Recurrent pregnancy loss]] | ||
! colspan="2" |[[Uterine]] abnormality | ! colspan="2" |[[Uterine]] abnormality | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | ![[Ultrasound]] | ||
! | !- | ||
|- | |- | ||
! colspan="2" |[[Chromosomal abnormality]] | ! colspan="2" |[[Chromosomal abnormality]] | ||
! | !- | ||
! | !-/+ | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | !- | ||
! | ![[Karyotyping]] | ||
! | !- | ||
|- | |- | ||
! colspan="2" |[[Hypothyroidism]] | ! colspan="2" |[[Hypothyroidism]] | ||
!- | |||
!-/+ | |||
!- | |||
!- | |||
![[Myxedema]] | |||
Dry skin | |||
!- | |||
!- | |||
!- | |||
!+ | |||
!- | |||
!- | |||
!- | |||
! | ! | ||
* Low [[T3]],[[T4]] | |||
* Normal/ low [[Thyroid-stimulating hormone|TSH]] | |||
* Rest of [[pituitary]] hormone levels WNL | |||
!- | |||
! | ! | ||
* Assays for anti-TPO and anti-Tg Ab | |||
! | ! | ||
* Dry skin | |||
* [[Bradycardia]] | |||
* Hair loss | |||
* [[Myxedema]] | |||
* Delayed relaxation phase of deep [[Tendon reflex|tendon reflexes]] | |||
|- | |- | ||
|} | |} |
Latest revision as of 20:32, 20 February 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]
Overview
Antiphospholipid syndrome should be differentiated from conditions such as hemolytic uremic syndrome-thrombotic thrombocytopenic purpura (HUS-TTP), disseminated intravascular coagulation (DIC), paroxysmal nocturnal hemoglobinuria, SLE, HELLP syndrome and other myeloproliferative diseases.
Differentiating Antiphospholipid antibody syndrome from other Diseases
- Hemolytic uremic syndrome-Thrombotic thrombocytopenic purpura (HUS-TTP)
- Disseminated Intravascular Coagulation (DIC)
- paroxysmal nocturnal hemoglobinuria
- SLE
- HELLP syndrome
- Myeloproliferative diseases
Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure, PCR= Polymerase chain reaction, PFT= Pulmonary function test.
Diseases | Clinical manifestations | Para-clinical findings | Gold standard | Additional findings | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Symptoms | Physical examination | |||||||||||||||||
Lab Findings | Imaging | Histopathology | ||||||||||||||||
Dyspnea | Fever | Weight loss | Arthralgia | Skin manifestation | Organomegaly | Focal neurological disorder | CBC | ESR | Antibodies | Other | CT scan | Other | ||||||
Antiphospholipid syndrome | + | + | - | - | Livedo reticularis, palpable purpura, leg ulcers, raynaud phenomena | hepatomegaly, | Stroke, transient ischemic attack | Schistocytes on peripheral smear,Thrombocytopenia, Hemolytic anemia | ↑ | Lupus anticoagulant,anticardiolipin antibodies | - | Stroke, | Doppler ultrasonography for recurrent DVT | Noninflammatory bland thrombosis without perivascular inflammation | Hx of thrombosis and antiphospholipid antibodies | Miscarriage, Pulmonary hypertension | ||
Systemic lupus erythematosus (SLE) | + | Leukopenia, Lymphopenia, Anemia, Thrombocytopenia | ↑ | Anti dsDNA, ANA | ↑Cr or BUN,
↑ALT or AST, Proteinuria |
Interstitial lung disease, Pneumonitis, Pulmonary emboli, Alveolar hemorrhage | Central nervous system (CNS) lupuswhite-matter changes in MRI | Staging lupus nephritis | Anti-dsDNA antibody test | Skin rashes or photosensitivity | ||||||||
Thrombosis | Thrombophilia | DIC | + | + | - | +/- | Acral cyanosis, Hemorrhagic skin infarctions | - | + | Thrombocytopenia, Schistocytes | ↑ | - | ↑D-dimer, aPTT and PT | Intracranial hemorrhage | - | Ischemia and necrosis due to fibrin deposition in small and medium-sized vessels | Clinical findings coupled with laboratory abnormalities | - |
HUS | + | + | - | + | Liver/spleen swelling | + | Anemia, | ↑ | ADAMTS13 | Increased lactate dehydrogenase | Thalami, brainstem, or cerebellumabnormality | Microthrombosesinclude fibrin thrombi that may occlude the glomerular tufts | Clinical findings coupled with laboratory abnormalities | Hematuria, Proteinuria | ||||
ITP | +/- | - | + | Petechiae | Splenomegaly | + | Anemia, Thrombocytopenia | - | Anti platelet factor 4 antibodies | HIV, ANA | R/O other causes | - | Increased number of normal morphologic megakaryocytes | Clinical findings coupled with thrombocytopenia | Easy bruising, Purpura | |||
HELLP | - | - | - | - | Hepatomegaly | + | Anemia, Thrombocytopenia, Schistocytes | - | - | ↑Bilirubin, ↓Haptoglobin, ↑LDH, ↑Cr | Cortical hypodense areas in the occipital lobes, Diffuse cerebral edema | Ultrasonography shows Poor fetal growth, Oligohydramnios, Abnormal umbilical artery | - | 24-hour urine study | Seizure, Edema | |||
Paroxysmal nocturnal hemoglobinuria (PNH) | + | + | - | +/- | Anemia, reticulocytosis,
Increased lactate dehydrogenase (LDH) and bilirubin |
- | Direct antiglobulin (Coombs) testing (DAT) | Increased haptoglobin | - | - | Bone marrow biopsy shows normocellular or hypercellular bone marrow with erythroid hyperplasia | Flow cytometry | ||||||
Heparin-induced thrombocytopenia (HIT) | + | + | - | - |
|
- | + | Thrombocytopenia | + | Anti-heparin-PF4 IgG |
|
Stroke, pulmonary embolism, mesenteric ischemia, intraabdominal or retroperitoneal bleeding, or acute limb ischemia. | - | Schistocytes | Serotonin release assay | Limb gangrene
Skin necrosis | ||
Myeloproliferative diseases | + | + | + | + |
|
Splenomegaly | - | ↑WBCs, predominantly neutrophils | + | - |
|
PET scan- metastasis in bone marrow | Terminal myeloid cell expansion | Philedelphia chromosome by PCR or BCR/ABL by FISH | ||||
Recurrent pregnancy loss | Uterine abnormality | - | - | - | - | - | - | - | - | - | - | - | - | - | - | Ultrasound | - | |
Chromosomal abnormality | - | -/+ | - | - | - | - | - | - | - | - | - | - | - | - | Karyotyping | - | ||
Hypothyroidism | - | -/+ | - | - | Myxedema
Dry skin |
- | - | - | + | - | - | - |
|
- |
|
|