Splenomegaly

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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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