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Revision as of 19:25, 27 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 xray	Echocardiography	Pulmonary edema Hyponatremia Elevated BNP
	Thrombosis of portal, hepatic, or splenic veins^[3]
Malignancy	Lymphoma^[4]
	Leukemia^[5]
	Polycythemia Vera^[6]
	Multiple myeloma^[7]
	Essential thrombocythemia^[8]
	Primary myelofibrosis^[9]
	Primary splenic tumors^[10]
	Metastatic solid tumors^[11]
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
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
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

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 55: / Line 55: @@
 |-
 ! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Congestive
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Cirrhosis]]<nowiki><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></nowiki>
+! 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
@@ Line 103: / Line 103: @@
 * Dupuytren's contracture
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Congestive heart failure|Heart failure]]
+! 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
@@ Line 144: / Line 144: @@
 * Elevated BNP
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Thrombosis]] of [[portal]], [[Liver|hepatic]], or [[Splenic vein|splenic veins]]
+! 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;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 172: / Line 172: @@
 |-
 ! rowspan="8" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Malignancy
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Lymphoma]]
+! colspan="2" align="center" style="background:#DCDCDC;" |[[Lymphoma]]<ref name="pmid25131182">{{cite journal |vauthors=Ishibashi N, Maebayashi T, Aizawa T, Sakaguchi M, Abe O, Saito T, Tanaka Y |title=Myelosuppression toxicity of palliative splenic irradiation in myelofibrosis and malignant lymphoma |journal=Hematology |volume=20 |issue=4 |pages=203–7 |date=May 2015 |pmid=25131182 |doi=10.1179/1607845414Y.0000000192 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 199: / Line 199: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Leukemia]]
+! 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;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 226: / Line 226: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Polycythemia vera|Polycythemia Vera]]
+! colspan="2" align="center" style="background:#DCDCDC;" |[[Polycythemia vera|Polycythemia Vera]]<ref name="pmid23281576">{{cite journal |vauthors=Chou YS, Gau JP, Yu YB, Pai JT, Hsiao LT, Liu JH, Hong YC, Liu CY, Yang CF, Chen PM, Chiou TJ, Tzeng CH |title=Leukocytosis in polycythemia vera and splenomegaly in essential thrombocythemia are independent risk factors for hemorrhage |journal=Eur. J. Haematol. |volume=90 |issue=3 |pages=228–36 |date=March 2013 |pmid=23281576 |doi=10.1111/ejh.12064 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 253: / Line 253: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Multiple myeloma]]
+! colspan="2" align="center" style="background:#DCDCDC;" |[[Multiple myeloma]]<ref name="pmid3400674">{{cite journal |vauthors=Grieco A, Manna R, Mancini R, Gambassi G |title=Massive hepatomegaly following splenectomy for myeloid metaplasia |journal=Am. J. Med. |volume=84 |issue=4 |pages=797 |date=April 1988 |pmid=3400674 |doi= |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 280: / Line 280: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Essential thrombocytosis|Essential thrombocythemia]]
+! colspan="2" align="center" style="background:#DCDCDC;" |[[Essential thrombocytosis|Essential thrombocythemia]]<ref name="pmid25611051">{{cite journal |vauthors=Tefferi A, Barbui T |title=Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management |journal=Am. J. Hematol. |volume=90 |issue=2 |pages=162–73 |date=February 2015 |pmid=25611051 |doi=10.1002/ajh.23895 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 307: / Line 307: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Myelofibrosis|Primary myelofibrosis]]
+! 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>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 334: / Line 334: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Splenic tumor|Primary splenic tumors]]
+! 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;" |
@@ Line 361: / Line 361: @@
 | align="center" style="background:#F5F5F5;" |
 |-
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Metastasis|Metastatic solid tumors]]
+! colspan="2" align="center" style="background:#DCDCDC;" |[[Metastasis|Metastatic solid tumors]]<ref name="pmid24172780">{{cite journal |vauthors=Huprikar NA, Kurtz MT, Mount CA |title=Massive splenomegaly and lymphopenia: a unique case of obstructive shock |journal=BMJ Case Rep |volume=2013 |issue= |pages= |date=October 2013 |pmid=24172780 |pmc=3822246 |doi=10.1136/bcr-2013-201643 |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 882: / Line 882: @@
 |-
 ! rowspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Infiltrative
-! colspan="2" align="center" style="background:#DCDCDC;" |[[Gaucher's disease|Gaucher disease]]
+! colspan="2" align="center" style="background:#DCDCDC;" |[[Gaucher's disease|Gaucher disease]]<ref name="pmid23510067">{{cite journal |vauthors=Ayto R, Hughes DA |title=Gaucher disease and myeloma |journal=Crit Rev Oncog |volume=18 |issue=3 |pages=247–68 |date=2013 |pmid=23510067 |doi= |url=}}</ref>
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |