Splenomegaly: Difference between revisions

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Revision as of 20:55, 29 September 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Gichoya Judy Wawira [2], Moi University School of Medicine

Synonyms and keywords: Spleen enlargement; enlarged spleen; spleen swelling

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Splenomegaly from Other Diseases

Category	Disease		Etiology	Clinical manifestations												Para−clinical findings										Gold standard	Associated findings
				Demography	History	Symptoms					Signs					Lab Findings									Imaging
						Fever	Fatigue	Weight	Abdominal pain	Bleeding	BP	JVP	Jaundice	Ascites	Other	CBC			Iron	LFT	PT/PTT	BUN/Cr	UA	Histopathology
						Fever	Fatigue	Weight	Abdominal pain	Bleeding	BP	JVP	Jaundice	Ascites	Other	WBC	Hb	Plt	Iron	LFT	PT/PTT	BUN/Cr	UA	Histopathology
Congestive	Cirrhosis^[1]		Infection Alcohol Autoimmune	Any, more in elderly	Hepatitis Alcohol use NAFLD	–	+	↑↓	+	+	↓	Nl	+	+	Spider angiomata Palmar erythema Weakness	↓	↓	↓	Nl	↑	↑	↑	Dark urine	Pancytopenia Regenerating nodules of hepatocytes Fibrosis Deposition of connective tissue between these nodules	Small and nodular liver on ultrasound Fibrosis on liver scan	Liver biopsy	Hepatic encephalopathy Gynecomastia Hypogonadism Esophageal varices Caput medusa Dupuytren's contracture
	Heart failure^[2]		Coronary artery disease HTN	Elderly	Smoking Sedentary lifestyle Obstructive sleep apnea	–	+	↑↓	–	–	↑	↑	±	+	Dyspnea Weakness Chest pain Heart murmur	↓	↓	↑	Nl	↑	↑	↑	Nl	NA	Normal or low ejection fraction on echocardiography Cardiomegaly on chest x-ray	Echocardiography	Pulmonary edema Hyponatremia Elevated BNP
	Thrombosis of portal, hepatic, or splenic veins^[3]		Thrombosis	Any	Inflammation Infection Cirrhosis Cancer	+	+	↑	+	+	↓	Nl	±	+	Weakness Hematemesis Melena	↓	↓	↓	Nl	↑	↑	↑	Dark urine	NA	Reduced or absent blood flow on doppler ultrasonography	Doppler ultrasonography	Portal hypertension Esophageal varices
Malignancy	Lymphoma^[4]		Unknown Mutation Infection	Any, most common malignant splenic tumor	Epstein Barr virus Positive family history Smoking HIV Autoimmune diseases	+	+	↓	+	+	Nl	Nl	+	–	Lymphadenopathy Itching Night sweats Anorexia Dyspnea	↓	↓	↓	High ferritin	↑	↑	↑	Nl	Different histopathological features based on types and stages Reed-Sternberg cell Malignant cells	Peritoneal lymphomatosis on CT scan	Lymph node biopsy	Primary immune disorder Post-transplant
	Leukemia^[5]		Unknown Mutation Bone marrowinfiltration Ineffective hematopoiesis Immune mediated	Elderly, male>female	History of hematological disorder History of chemotherapy andionizing radiation	+	+	↓	+	+	Nl	Nl	±	+	Pallor Leukemia cutis Bruising Petechiae Ecchymosis Fatigue Bone tenderness Early satiety	↓	↓	↓	High ferritin	↑	↑	↑	Nl	Leukemic blasts Leukoerythroblastic features Giant platelets	NA	Bone marrow examination + clinical manifestation	Infections of variable severity Cranial nerve palsy
	Polycythemia Vera^[6]		Autonomous erythrocyteproduction JAK2mutation	Mean age >60 years old	Positive family history	–	+	Nl	+	–	Nl	Nl	–	–	Erythromelalgia Chest pain Stroke-like symptoms Pruritus Dizziness Visual disturbance Facial plethora Painful erythema Lymphadenopathy	Nl to ↑	↑	Nl to ↑	High ferritin	Nl	Nl	Nl	Nl	Elevated normochromic, normocytic RBCs Thrombocytosis Rarely immature cells Leukoerythroblastic picture	NA	WHO criteria for PV	Stroke Venous thrombosis Myelofibrosis Acute leukemia
	Multiple myeloma^[7]		Unknown Mutation Infection	Any, more in adults	Exposure to chemicals Radiation Pre-existent blood disorders	+	+	↓	+	+	Nl	Nl	+	–	Lymphadenopathy Itching Night sweats Anorexia Dyspnea	↓	↓	↓	High ferritin	Nl	↑	↑	Hemoglobinuria	Blast cells		Bone marrow examination	Tumor lysis syndrome Infection CNS involvement DIC
	Essential thrombocythemia^[8]		Acquired mutation of JAK2, CALR, or MPL	Mean age >60 years old, female > male	Vascular occlusive events and hemorrhages	±	+	Nl	+	+	Nl	Nl	–	–	Flushing Erythromelalgia Transient ischemic attacks Priapism Decreased visual acuity	↑	↑	↑↑	Nl	Nl	Nl	Nl	Nl	Increased cellularity Hyperplasia and clustering of megakaryocytes Bone marrow reticulin may be increased with no collagen fibrosis	Splenomegaly on abdominal CT VTE on spiral chest CT DVT on ultrasound	Bone marrow biopsy	Thrombosis
	Primary myelofibrosis^[9]		Bone marrow fibrosis Mutation in JAK2, CALR, or MPL	Mean age >60 years old, male> female	Other myeloproliferativedisorders Radiation exposure Chemicals exposure	–	+	Nl	+	+	Nl	Nl	+	–	Pallor Bruising Skin nodules Insert paragraph Fatigue Abdominal pain Early satiety Dyspnea Lymphadenopathy	↓	↓	↑/↓	Low iron	Nl	↑	Nl	Dark urine	Proliferation and atypia of megakaryocytes Reticulin or collagen fibrosis Hypercellular bone marrow	Diffuse bone sclerosis on CT	Bone marrow biopsy	Thrombosis Variable risk for development of acute leukemia
	Primary splenic tumors^[10]			Rare
	Metastatic solid tumors^[11]		Bone marrowinfiltration Ineffective hematopoiesis Immune mediated	Any, more in adults	Exposure to chemicals Radiation Pre-existent blood disorders	+	+	↓	+	+	↓	Nl	±	±	Petechiae Weakness	↓	↓	↓	Low iron, high ferritin	↑	↑	↑	Dark urine	Blast cells		Biopsy	Tumor lysis syndrome Infection CNS involvement DIC
Category	Disease		Etiology	Demography	History	Fever	Fatigue	Weight	Abdominal pain	Bleeding	BP	JVP	Jaundice	Ascites	Other	WBC	Hb	Plt	Iron	LFT	PT/PTT	BUN/Cr	UA	Histopathology	Imaging	Gold standard	Associated findings
Infection	Viral	Hepatitis
		Infectious mononucleosis
		Cytomegalovirus
	Bacterial	Salmonella
		Brucella
		Tuberculosis
	Parasitic	Malaria
		Schistosomiasis
		Toxoplasmosis
		Kala-azar	Visceral leishmaniasis
	Infective endocarditis
	Fungal
Inflammation	Sarcoidosis
	Serum sickness
	Systemic lupus erythematosus
	Felty syndrome
Category	Disease		Etiology	Demography	History	Fever	Fatigue	Weight loss	Abdominal pain	Bleeding	BP	JVP	Jaundice	Ascites	Other	WBC	Hb	Plt	Iron	LFT	PT/PTT	BUN/Cr	UA	Histopathology	Imaging	Gold standard	Associated findings
Infiltrative	Gaucher disease^[12]
	Niemann-Pick disease
	Amyloidosis
	Langerhans cell histiocytosis
	Hemophagocytic lymphohistiocytosis
	Rosai-Dorfman disease
Hematologic (hypersplenic) states	Hemolytic anemia
	Sickle cell disease
	Granulocyte colony-stimulating factor

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Treatment

Case Studies

Case #1

Related Chapters

References

↑ Wu Z, Zhou J, Pankaj P, Peng B (December 2012). "Laparoscopic and open splenectomy for splenomegaly secondary to liver cirrhosis: an evaluation of immunity". Surg Endosc. 26 (12): 3557–64. doi:10.1007/s00464-012-2366-5. PMID 22710653.
↑ Gedela M, Khan M, Jonsson O (September 2015). "Heart Failure". S D Med. 68 (9): 403–5, 407–9. PMID 26489162.
↑ Ferri PM, Ferreira AR, Fagundes ED, Liu SM, Roquete ML, Penna FJ (2012). "Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center". Arq Gastroenterol. 49 (1): 69–76. PMID 22481689.
↑ Ishibashi N, Maebayashi T, Aizawa T, Sakaguchi M, Abe O, Saito T, Tanaka Y (May 2015). "Myelosuppression toxicity of palliative splenic irradiation in myelofibrosis and malignant lymphoma". Hematology. 20 (4): 203–7. doi:10.1179/1607845414Y.0000000192. PMID 25131182.
↑ Quest GR, Johnston JB (December 2015). "Clinical features and diagnosis of hairy cell leukemia". Best Pract Res Clin Haematol. 28 (4): 180–92. doi:10.1016/j.beha.2015.10.017. PMID 26614896.
↑ Chou YS, Gau JP, Yu YB, Pai JT, Hsiao LT, Liu JH, Hong YC, Liu CY, Yang CF, Chen PM, Chiou TJ, Tzeng CH (March 2013). "Leukocytosis in polycythemia vera and splenomegaly in essential thrombocythemia are independent risk factors for hemorrhage". Eur. J. Haematol. 90 (3): 228–36. doi:10.1111/ejh.12064. PMID 23281576.
↑ Grieco A, Manna R, Mancini R, Gambassi G (April 1988). "Massive hepatomegaly following splenectomy for myeloid metaplasia". Am. J. Med. 84 (4): 797. PMID 3400674.
↑ Tefferi A, Barbui T (February 2015). "Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management". Am. J. Hematol. 90 (2): 162–73. doi:10.1002/ajh.23895. PMID 25611051.
↑ Cervantes F, Martinez-Trillos A (May 2013). "Myelofibrosis: an update on current pharmacotherapy and future directions". Expert Opin Pharmacother. 14 (7): 873–84. doi:10.1517/14656566.2013.783019. PMID 23514013.
↑ Adachi K, Ui M, Nojima H, Takada Y, Enatsu K (August 2011). "Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy". Am. J. Surg. 202 (2): e17–9. doi:10.1016/j.amjsurg.2010.10.002. PMID 21810495.
↑ Huprikar NA, Kurtz MT, Mount CA (October 2013). "Massive splenomegaly and lymphopenia: a unique case of obstructive shock". BMJ Case Rep. 2013. doi:10.1136/bcr-2013-201643. PMC 3822246. PMID 24172780.
↑ Ayto R, Hughes DA (2013). "Gaucher disease and myeloma". Crit Rev Oncog. 18 (3): 247–68. PMID 23510067.

[pmid22710653-1] Wu Z, Zhou J, Pankaj P, Peng B (December 2012). "Laparoscopic and open splenectomy for splenomegaly secondary to liver cirrhosis: an evaluation of immunity". Surg Endosc. 26 (12): 3557–64. doi:10.1007/s00464-012-2366-5. PMID 22710653.

[pmid26489162-2] Gedela M, Khan M, Jonsson O (September 2015). "Heart Failure". S D Med. 68 (9): 403–5, 407–9. PMID 26489162.

[pmid22481689-3] Ferri PM, Ferreira AR, Fagundes ED, Liu SM, Roquete ML, Penna FJ (2012). "Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center". Arq Gastroenterol. 49 (1): 69–76. PMID 22481689.

[pmid25131182-4] Ishibashi N, Maebayashi T, Aizawa T, Sakaguchi M, Abe O, Saito T, Tanaka Y (May 2015). "Myelosuppression toxicity of palliative splenic irradiation in myelofibrosis and malignant lymphoma". Hematology. 20 (4): 203–7. doi:10.1179/1607845414Y.0000000192. PMID 25131182.

[pmid26614896-5] Quest GR, Johnston JB (December 2015). "Clinical features and diagnosis of hairy cell leukemia". Best Pract Res Clin Haematol. 28 (4): 180–92. doi:10.1016/j.beha.2015.10.017. PMID 26614896.

[pmid23281576-6] Chou YS, Gau JP, Yu YB, Pai JT, Hsiao LT, Liu JH, Hong YC, Liu CY, Yang CF, Chen PM, Chiou TJ, Tzeng CH (March 2013). "Leukocytosis in polycythemia vera and splenomegaly in essential thrombocythemia are independent risk factors for hemorrhage". Eur. J. Haematol. 90 (3): 228–36. doi:10.1111/ejh.12064. PMID 23281576.

[pmid3400674-7] Grieco A, Manna R, Mancini R, Gambassi G (April 1988). "Massive hepatomegaly following splenectomy for myeloid metaplasia". Am. J. Med. 84 (4): 797. PMID 3400674.

[pmid25611051-8] Tefferi A, Barbui T (February 2015). "Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management". Am. J. Hematol. 90 (2): 162–73. doi:10.1002/ajh.23895. PMID 25611051.

[pmid23514013-9] Cervantes F, Martinez-Trillos A (May 2013). "Myelofibrosis: an update on current pharmacotherapy and future directions". Expert Opin Pharmacother. 14 (7): 873–84. doi:10.1517/14656566.2013.783019. PMID 23514013.

[pmid21810495-10] Adachi K, Ui M, Nojima H, Takada Y, Enatsu K (August 2011). "Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy". Am. J. Surg. 202 (2): e17–9. doi:10.1016/j.amjsurg.2010.10.002. PMID 21810495.

[pmid24172780-11] Huprikar NA, Kurtz MT, Mount CA (October 2013). "Massive splenomegaly and lymphopenia: a unique case of obstructive shock". BMJ Case Rep. 2013. doi:10.1136/bcr-2013-201643. PMC 3822246. PMID 24172780.

[pmid23510067-12] Ayto R, Hughes DA (2013). "Gaucher disease and myeloma". Crit Rev Oncog. 18 (3): 247–68. PMID 23510067.

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@@ Line 57: / Line 57: @@
 ! colspan="2" align="center" style="background:#DCDCDC;" |[[Cirrhosis]]<ref name="pmid22710653">{{cite journal |vauthors=Wu Z, Zhou J, Pankaj P, Peng B |title=Laparoscopic and open splenectomy for splenomegaly secondary to liver cirrhosis: an evaluation of immunity |journal=Surg Endosc |volume=26 |issue=12 |pages=3557–64 |date=December 2012 |pmid=22710653 |doi=10.1007/s00464-012-2366-5 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
-* Infection
+* [[Infection]]
-* Alcohol
+* [[Alcohol]]
-* Autoimmune
+* [[Autoimmunity|Autoimmune]]
 | align="center" style="background:#F5F5F5;" |Any, more in elderly
 | align="center" style="background:#F5F5F5;" |
@@ Line 75: / Line 75: @@
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |
-* Spider angiomata
+* [[Spider angioma|Spider angiomata]]
-* Palmar erythema
+* [[Palmar erythema]]
-* Weakness
+* [[Muscle weakness|Weakness]]
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
@@ Line 86: / Line 86: @@
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |
-* Dark urine
+* Dark [[urine]]
 | align="center" style="background:#F5F5F5;" |
-* Pancytopenia
+* [[Pancytopenia]]
-* Regenerating nodules of hepatocytes
+* Regenerating nodules of [[Hepatocyte|hepatocytes]]
-* Fibrosis
+* [[Fibrosis]]
-* Deposition of connective tissue between these nodules
+* Deposition of [[connective tissue]] between these nodules
 | align="center" style="background:#F5F5F5;" |
-* Small and nodular liver on ultrasound
+* Small and nodular [[liver]] on [[ultrasound]]
-* Fibrosis on liver scan
+* [[Fibrosis]] on [[liver]] scan
-| align="center" style="background:#F5F5F5;" |Liver biopsy
+| align="center" style="background:#F5F5F5;" |[[Liver biopsy]]
 | align="center" style="background:#F5F5F5;" |
-* Hepatic encephalopathy
+* [[Hepatic encephalopathy]]
-* Gynecomastia
+* [[Gynecomastia]]
-* Hypogonadism
+* [[Hypogonadism]]
-* Esophageal varices
+* [[Esophageal varices]]
-* Caput medusa
+* [[Caput medusae|Caput medusa]]
-* Dupuytren's contracture
+* [[Dupuytrens contracture|Dupuytren's contracture]]
 |-
 ! colspan="2" align="center" style="background:#DCDCDC;" |[[Congestive heart failure|Heart failure]]<ref name="pmid26489162">{{cite journal |vauthors=Gedela M, Khan M, Jonsson O |title=Heart Failure |journal=S D Med |volume=68 |issue=9 |pages=403–5, 407–9 |date=September 2015 |pmid=26489162 |doi= |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
-* Coronary artery disease
+* [[Coronary heart disease|Coronary artery disease]]
-* HTN
+* [[Hypertension|HTN]]
 | align="center" style="background:#F5F5F5;" |Elderly
 | align="center" style="background:#F5F5F5;" |
-* Smoking
+* [[Smoking]]
 * Sedentary lifestyle
-* Obstructive sleep apnea
+* [[Sleep apnea|Obstructive sleep apnea]]
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" | +
@@ Line 123: / Line 123: @@
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |
-* Dyspnea
+* [[Dyspnea]]
-* Weakness
+* [[Muscle weakness|Weakness]]
-* Chest pain
+* [[Chest pain]]
-* Heart murmur
+* [[Heart murmur]]
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
@@ Line 137: / Line 137: @@
 | align="center" style="background:#F5F5F5;" |NA
 | align="center" style="background:#F5F5F5;" |
-* Normal or low ejection fraction on echocardiography
+* Normal or low [[ejection fraction]] on [[echocardiography]]
-* Cardiomegaly on chest xray
+* [[Cardiomegaly]] on [[Chest X-ray|chest x-ray]]
-| align="center" style="background:#F5F5F5;" |Echocardiography
+| align="center" style="background:#F5F5F5;" |[[Echocardiography]]
 | align="center" style="background:#F5F5F5;" |
-* Pulmonary edema
+* [[Pulmonary edema]]
-* Hyponatremia
+* [[Hyponatremia]]
-* Elevated BNP
+* Elevated [[Brain natriuretic peptide|BNP]]
 |-
 ! colspan="2" align="center" style="background:#DCDCDC;" |[[Thrombosis]] of [[portal]], [[Liver|hepatic]], or [[Splenic vein|splenic veins]]<ref name="pmid22481689">{{cite journal |vauthors=Ferri PM, Ferreira AR, Fagundes ED, Liu SM, Roquete ML, Penna FJ |title=Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center |journal=Arq Gastroenterol |volume=49 |issue=1 |pages=69–76 |date=2012 |pmid=22481689 |doi= |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
-* Thrombosis
+* [[Thrombosis]]
 | align="center" style="background:#F5F5F5;" |Any
 | align="center" style="background:#F5F5F5;" |
-* Inflammation
+* [[Inflammation]]
-* Infection
+* [[Infection]]
-* Cirrhosis
+* [[Cirrhosis]]
-* Cancer
+* [[Cancer]]
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
@@ Line 164: / Line 164: @@
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |
-* Weakness
+* [[Muscle weakness|Weakness]]
-*Hematemesis
+*[[Hematemesis]]
-*Melena
+*[[Melena]]
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
@@ Line 175: / Line 175: @@
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |
-* Dark urine
+* Dark [[urine]]
 | align="center" style="background:#F5F5F5;" |NA
 | align="center" style="background:#F5F5F5;" |
-* Reduced or absent blood flow on doppler ultrasonography
+* Reduced or absent [[blood flow]] on [[Medical ultrasonography|doppler ultrasonography]]
-| align="center" style="background:#F5F5F5;" |Doppler ultrasonography
+| align="center" style="background:#F5F5F5;" |[[Medical ultrasonography|Doppler ultrasonography]]
 | align="center" style="background:#F5F5F5;" |
-* Portal hypertension
+* [[Portal hypertension]]
-* Esophageal varices
+* [[Esophageal varices]]
 |-
 ! rowspan="8" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Malignancy
@@ Line 188: / Line 188: @@
 | align="center" style="background:#F5F5F5;" |
 * Unknown
-* Mutation
+* [[Mutation]]
-* Infection
+* [[Infection]]
-| align="center" style="background:#F5F5F5;" |Any
+| align="center" style="background:#F5F5F5;" |Any, most common malignant splenic tumor
 | align="center" style="background:#F5F5F5;" |
-* Epstein–Barr virus
+* [[Epstein Barr virus]]
 * Positive family history
-* Smoking
+* [[Smoking]]
-* HIV
+* [[Human Immunodeficiency Virus (HIV)|HIV]]
-* Autoimmune diseases
+* [[Autoimmunity|Autoimmune diseases]]
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
@@ Line 207: / Line 207: @@
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" |
-* Lymphadenopathy
+* [[Lymphadenopathy]]
-* Itching
+* [[Itch|Itching]]
-* Night sweats
+* [[Sleep hyperhidrosis|Night sweats]]
-* Anorexia
+* [[Anorexia]]
-* Respiratory distress or dyspnea
+* [[Dyspnea]]
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |High ferritin
+| align="center" style="background:#F5F5F5;" |High [[ferritin]]
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
@@ Line 221: / Line 221: @@
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |
-* Different histopathological features based on types and stages
+* Different [[Histopathology|histopathological]] features based on types and stages
-* Reed–Sternberg cell
+* [[Reed-Sternberg cell]]
 * Malignant cells
 | align="center" style="background:#F5F5F5;" |
-* Peritoneal lymphomatosis on CT scan
+* Peritoneal [[Primary central nervous system lymphoma|lymphomatosis]] on [[Computed tomography|CT scan]]
-| align="center" style="background:#F5F5F5;" |Lymph node biopsy
+| align="center" style="background:#F5F5F5;" |[[Lymph node biopsy]]
 | align="center" style="background:#F5F5F5;" |
-* Primary immune disorder
+* [[Immune disorder|Primary immune disorder]]
-* Post-transplant
+* Post-[[Organ transplant|transplant]]
 |-
 ! colspan="2" align="center" style="background:#DCDCDC;" |[[Leukemia]]<ref name="pmid26614896">{{cite journal |vauthors=Quest GR, Johnston JB |title=Clinical features and diagnosis of hairy cell leukemia |journal=Best Pract Res Clin Haematol |volume=28 |issue=4 |pages=180–92 |date=December 2015 |pmid=26614896 |doi=10.1016/j.beha.2015.10.017 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 * Unknown
-* Mutation
+* [[Mutation]]
 * [[Bone marrow]]<nowiki/>infiltration
 * Ineffective [[hematopoiesis]]
-* Immune mediated
+* [[Immunity (medical)|Immune]] mediated
 | align="center" style="background:#F5F5F5;" |Elderly, male>female
 | align="center" style="background:#F5F5F5;" |
-* History of hematological disorder
+* History of [[Hematological|hematological disorder]]
 * History of [[chemotherapy]] and[[ionizing radiation]]
 | align="center" style="background:#F5F5F5;" | +
@@ Line 261: / Line 261: @@
 * [[Fatigue]]
 * Bone [[tenderness]]
-* Early satiety
+* [[Satiety|Early satiety]]
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |High ferritin
+| align="center" style="background:#F5F5F5;" |High [[ferritin]]
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
@@ Line 297: / Line 297: @@
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" |
-* Erythromelagia
+* [[Erythromelalgia]]
-* Chest pain
+* [[Chest pain]]
-* Stroke-like symptoms
+* [[Stroke]]-like symptoms
-* Pruritis
+* [[Itch|Pruritus]]
-* Dizziness
+* [[Dizziness]]
-* Visual disturbance
+* [[Visual system|Visual disturbance]]
-* Facial plethora
+* Facial [[Hypervolemia|plethora]]
-* Painful erythema
+* Painful [[erythema]]
-* Lymphadenopathy
+* [[Lymphadenopathy]]
 | align="center" style="background:#F5F5F5;" |Nl to ↑
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |Nl to ↑
-| align="center" style="background:#F5F5F5;" |High ferritin
+| align="center" style="background:#F5F5F5;" |High [[ferritin]]
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |Nl
@@ Line 320: / Line 320: @@
 * Leukoerythroblastic picture
 | align="center" style="background:#F5F5F5;" |NA
-| align="center" style="background:#F5F5F5;" |WHO criteria for PV
+| align="center" style="background:#F5F5F5;" |WHO criteria for [[Polycythemia vera|PV]]
 | align="center" style="background:#F5F5F5;" |
 * [[Stroke]]
@@ Line 330: / Line 330: @@
 | align="center" style="background:#F5F5F5;" |
 * Unknown
-* Mutation
+* [[Mutation]]
-* Infection
+* [[Infection]]
 | align="center" style="background:#F5F5F5;" |Any, more in adults
 | align="center" style="background:#F5F5F5;" |
 * Exposure to chemicals
 * [[Radiation therapy|Radiation]]
-* Pre-existent blood disorders
+* Pre-existent [[Hematology|blood disorders]]
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
@@ Line 347: / Line 347: @@
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" |
-* Lymphadenopathy
+* [[Lymphadenopathy]]
-* Itching
+* [[Itch|Itching]]
-* Night sweats
+* [[Sleep hyperhidrosis|Night sweats]]
-* Anorexia
+* [[Anorexia]]
-* Dyspnea
+* [[Dyspnea]]
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |High ferritin
+| align="center" style="background:#F5F5F5;" |High [[ferritin]]
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |
-* Hemoglobinuria
+* [[Hemoglobinuria]]
 | align="center" style="background:#F5F5F5;" |
 * Blast cells
 | align="center" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |Bone marrow examination
+| align="center" style="background:#F5F5F5;" |[[Bone marrow examination]]
 | align="center" style="background:#F5F5F5;" |
 * [[Tumor lysis syndrome]]
@@ Line 387: / Line 387: @@
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" |
-* Flushing
+* [[Flushing]]
-* Erythromelalgia
+* [[Erythromelalgia]]
-* Transient ischemic attacks
+* [[Transient ischemic attack|Transient ischemic attacks]]
-* Priapism
+* [[Priapism]]
-* Decreased visual acuity
+* [[Blurred vision|Decreased visual acuity]]
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
@@ Line 402: / Line 402: @@
 | align="center" style="background:#F5F5F5;" |
 * Increased cellularity
-* Hyperplasia and clustering of megakaryocytes
+* [[Hyperplasia]] and clustering of [[Megakaryocyte|megakaryocytes]]
-* Bone marrow reticulin may be increased with no collagen fibrosis
+* [[Bone marrow]] [[Reticular fiber|reticulin]] may be increased with no [[collagen]] [[fibrosis]]
 | align="center" style="background:#F5F5F5;" |
-* Splenomegaly on abdominal CT
+* [[Splenomegaly]] on abdominal CT
 * [[VTE]] on spiral chest CT
-* [[Deep vein thrombosis|DVT]] on ultrasound
+* [[Deep vein thrombosis|DVT]] on [[ultrasound]]
 | align="center" style="background:#F5F5F5;" |[[Bone marrow examination|Bone marrow biopsy]]
 | align="center" style="background:#F5F5F5;" |
-* Thrombo-hemorrhagic complications
+* [[Thrombosis]]
 |-
 ! colspan="2" align="center" style="background:#DCDCDC;" |[[Myelofibrosis|Primary myelofibrosis]]<ref name="pmid23514013">{{cite journal |vauthors=Cervantes F, Martinez-Trillos A |title=Myelofibrosis: an update on current pharmacotherapy and future directions |journal=Expert Opin Pharmacother |volume=14 |issue=7 |pages=873–84 |date=May 2013 |pmid=23514013 |doi=10.1517/14656566.2013.783019 |url=}}</ref>
@@ Line 420: / Line 420: @@
 * Other [[Myeloproliferative neoplasm|myeloproliferative]]<nowiki/>disorders
 * [[Radioactive contamination|Radiation exposure]]
-* Chemicals exposure
+* [[Chemical substance|Chemicals]] exposure
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |Nl
-| align="center" style="background:#F5F5F5;" |+
+| align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |+
+| align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |Nl
-| align="center" style="background:#F5F5F5;" |+
+| align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |–
 | align="center" style="background:#F5F5F5;" |
@@ Line 444: / Line 444: @@
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↑/↓
-| align="center" style="background:#F5F5F5;" |Low iron
+| align="center" style="background:#F5F5F5;" |Low [[iron]]
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |
-* Dark urine
+* [[Urine|Dark urine]]
 | align="center" style="background:#F5F5F5;" |
-* Proliferation and atypia of [[megakaryocytes]]
+* Proliferation and [[atypia]] of [[megakaryocytes]]
 * Reticulin or [[Collagen|collagen fibrosis]]
 * Hypercellular [[bone marrow]]
@@ Line 458: / Line 458: @@
 | align="center" style="background:#F5F5F5;" |[[Bone marrow examination|Bone marrow biopsy]]
 | align="center" style="background:#F5F5F5;" |
-* Thrombo-hemorrhagic complications
+* [[Thrombosis]]
 * Variable risk for development of [[acute leukemia]]
 |-
 ! colspan="2" align="center" style="background:#DCDCDC;" |[[Splenic tumor|Primary splenic tumors]]<ref name="pmid21810495">{{cite journal |vauthors=Adachi K, Ui M, Nojima H, Takada Y, Enatsu K |title=Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy |journal=Am. J. Surg. |volume=202 |issue=2 |pages=e17–9 |date=August 2011 |pmid=21810495 |doi=10.1016/j.amjsurg.2010.10.002 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |[[Rare]]
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 492: / Line 492: @@
 * [[Bone marrow]]<nowiki/>infiltration
 * Ineffective [[hematopoiesis]]
-* Immune mediated
+* [[Immunity (medical)|Immune]] mediated
 | align="center" style="background:#F5F5F5;" |Any, more in adults
 | align="center" style="background:#F5F5F5;" |
 * Exposure to chemicals
 * [[Radiation therapy|Radiation]]
-* Pre-existent blood disorders
+* Pre-existent [[Hematology|blood disorders]]
+| align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |+
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |+
+| align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |Nl
@@ Line 513: / Line 513: @@
 | align="center" style="background:#F5F5F5;" |↓
 | align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |Low iron, high ferritin
+| align="center" style="background:#F5F5F5;" |Low [[iron]], high [[ferritin]]
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |
-* Dark urine
+* [[Urine|Dark urine]]
 | align="center" style="background:#F5F5F5;" |
 * Blast cells
 | align="center" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |Biopsy
+| align="center" style="background:#F5F5F5;" |[[Biopsy]]
 | align="center" style="background:#F5F5F5;" |
 * [[Tumor lysis syndrome]]