Splenomegaly - Revision history

Danbot: Remove refs with incorrect PMIDs (verified against PubMed)

2026-03-22T10:25:06Z

Remove refs with incorrect PMIDs (verified against PubMed)

← Older revision		Revision as of 10:25, 22 March 2026
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	==Management==		==Management==
	*Treat the underlying cause~~<ref>Renzulli P, et al. Atraumatic splenic rupture: a systematic review. Int J Surg. 2009;7(4):261-268. PMID 19409517</ref>~~		*Treat the underlying cause
	*Splenic rupture: Hemodynamic resuscitation, emergent surgical consultation, massive transfusion protocol if indicated (see [[Splenic injury]])		*Splenic rupture: Hemodynamic resuscitation, emergent surgical consultation, massive transfusion protocol if indicated (see [[Splenic injury]])
	*Splenic infarction: Supportive care, analgesia, anticoagulation if embolic source identified; surgical consultation if concern for abscess formation or rupture		*Splenic infarction: Supportive care, analgesia, anticoagulation if embolic source identified; surgical consultation if concern for abscess formation or rupture

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2026-03-22T10:06:47Z

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← Older revision		Revision as of 10:06, 22 March 2026
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	==Management==		==Management==
	*Treat the underlying cause		*Treat the underlying cause<ref>Renzulli P, et al. Atraumatic splenic rupture: a systematic review. Int J Surg. 2009;7(4):261-268. PMID 19409517</ref>
	*Splenic rupture: Hemodynamic resuscitation, emergent surgical consultation, massive transfusion protocol if indicated (see [[Splenic injury]])		*Splenic rupture: Hemodynamic resuscitation, emergent surgical consultation, massive transfusion protocol if indicated (see [[Splenic injury]])
	*Splenic infarction: Supportive care, analgesia, anticoagulation if embolic source identified; surgical consultation if concern for abscess formation or rupture		*Splenic infarction: Supportive care, analgesia, anticoagulation if embolic source identified; surgical consultation if concern for abscess formation or rupture

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2026-03-22T09:30:18Z

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← Older revision		Revision as of 09:30, 22 March 2026
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	==Background==		==Background==
	*The spleen normally weighs 150-200g in adults and is not palpable on exam		*The spleen normally weighs 150-200g in adults and is not palpable on exam
	*A palpable spleen tip below the left costal margin generally indicates ~~'''~~at least 1.5-2× enlargement~~'''~~ — by the time a spleen is palpable, it is significantly enlarged		*A palpable spleen tip below the left costal margin generally indicates at least 1.5-2× enlargement — by the time a spleen is palpable, it is significantly enlarged
	*Massive splenomegaly (spleen extends beyond the umbilicus or >20 cm on imaging) has a narrower differential: chronic myeloproliferative disorders (especially CML), myelofibrosis, hairy cell leukemia, malaria, kala-azar (visceral leishmaniasis), Gaucher disease		*Massive splenomegaly (spleen extends beyond the umbilicus or >20 cm on imaging) has a narrower differential: chronic myeloproliferative disorders (especially CML), myelofibrosis, hairy cell leukemia, malaria, kala-azar (visceral leishmaniasis), Gaucher disease
	*Hypersplenism = splenomegaly + cytopenias (from splenic sequestration and destruction of blood cells) — may see anemia, thrombocytopenia, and/or leukopenia		*Hypersplenism = splenomegaly + cytopenias (from splenic sequestration and destruction of blood cells) — may see anemia, thrombocytopenia, and/or leukopenia
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	==Clinical Features==		==Clinical Features==
	===Symptoms===		===Symptoms===
	*Often ~~'''~~asymptomatic~~'''~~ — discovered incidentally		*Often asymptomatic — discovered incidentally
	*Left upper quadrant pain or fullness, early satiety (mass effect on stomach)		*Left upper quadrant pain or fullness, early satiety (mass effect on stomach)
	*Left shoulder pain (Kehr sign — referred diaphragmatic irritation from splenic pathology, classically splenic rupture or infarct)		*Left shoulder pain (Kehr sign — referred diaphragmatic irritation from splenic pathology, classically splenic rupture or infarct)
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	==Differential Diagnosis==		==Differential Diagnosis==
	===Infectious===		===Infectious===
	*Viral: [[EBV]] (mononucleosis — ~~'''~~most common infectious cause in young adults~~'''~~), [[CMV]], [[HIV]], viral [[hepatitis]]		*Viral: [[EBV]] (mononucleosis — most common infectious cause in young adults), [[CMV]], [[HIV]], viral [[hepatitis]]
	*Bacterial: [[Endocarditis]], [[tuberculosis]], [[brucellosis]], [[typhoid]], splenic abscess		*Bacterial: [[Endocarditis]], [[tuberculosis]], [[brucellosis]], [[typhoid]], splenic abscess
	*Parasitic: [[Malaria]] (massive splenomegaly in endemic areas), kala-azar (visceral leishmaniasis), [[schistosomiasis]]		*Parasitic: [[Malaria]] (massive splenomegaly in endemic areas), kala-azar (visceral leishmaniasis), [[schistosomiasis]]
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	===Congestive (Portal Hypertension)===		===Congestive (Portal Hypertension)===
	*[[Cirrhosis]] — ~~'''~~most common cause of splenomegaly in adults in developed countries~~'''~~		*[[Cirrhosis]] — most common cause of splenomegaly in adults in developed countries
	*Portal vein thrombosis		*Portal vein thrombosis
	*Splenic vein thrombosis		*Splenic vein thrombosis
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	===Autoimmune/Inflammatory===		===Autoimmune/Inflammatory===
	*[[Systemic lupus erythematosus]]		*[[Systemic lupus erythematosus]]
	*~~'''~~[[Adult-onset Still's disease]]~~'''~~ / ~~'''~~[[Systemic JIA]]~~'''~~		*[[Adult-onset Still's disease]] / [[Systemic JIA]]
	*[[Rheumatoid arthritis]] (Felty syndrome = RA + splenomegaly + neutropenia)		*[[Rheumatoid arthritis]] (Felty syndrome = RA + splenomegaly + neutropenia)
	*[[Sarcoidosis]]		*[[Sarcoidosis]]
	*[[Macrophage activation syndrome]] / ~~'''~~[[Hemophagocytic lymphohistiocytosis]]~~'''~~		*[[Macrophage activation syndrome]] / [[Hemophagocytic lymphohistiocytosis]]

	===Infiltrative/Storage Diseases===		===Infiltrative/Storage Diseases===
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	*Splenic abscess: IV antibiotics + interventional radiology or surgical drainage		*Splenic abscess: IV antibiotics + interventional radiology or surgical drainage
	*Splenic sequestration crisis (sickle cell): Aggressive IV fluids, transfusion (target Hgb rise of 1-2 g/dL — avoid over-transfusion which can cause auto-transfusion from the spleen and hyperviscosity), emergent hematology consultation		*Splenic sequestration crisis (sickle cell): Aggressive IV fluids, transfusion (target Hgb rise of 1-2 g/dL — avoid over-transfusion which can cause auto-transfusion from the spleen and hyperviscosity), emergent hematology consultation
	*Activity restriction: Counsel patients with newly diagnosed splenomegaly (especially EBV/mononucleosis) to ~~'''~~avoid contact sports and heavy lifting~~'''~~ until splenomegaly has resolved — risk of splenic rupture		*Activity restriction: Counsel patients with newly diagnosed splenomegaly (especially EBV/mononucleosis) to avoid contact sports and heavy lifting until splenomegaly has resolved — risk of splenic rupture

	==Disposition==		==Disposition==
	*Admit: Splenic rupture, splenic infarction with hemodynamic instability, splenic abscess, splenic sequestration crisis, new diagnosis of hematologic malignancy, unexplained massive splenomegaly with cytopenias, suspected MAS/HLH		*Admit: Splenic rupture, splenic infarction with hemodynamic instability, splenic abscess, splenic sequestration crisis, new diagnosis of hematologic malignancy, unexplained massive splenomegaly with cytopenias, suspected MAS/HLH
	*Discharge with urgent follow-up (24-72h): Hemodynamically stable patients with incidentally discovered mild-moderate splenomegaly and non-critical initial labs; ensure follow-up with hematology, hepatology, or primary care with pending workup communicated		*Discharge with urgent follow-up (24-72h): Hemodynamically stable patients with incidentally discovered mild-moderate splenomegaly and non-critical initial labs; ensure follow-up with hematology, hepatology, or primary care with pending workup communicated
	*Return precautions: LUQ pain, lightheadedness/syncope, worsening fatigue, fevers, bleeding/bruising; ~~'''~~avoid contact sports~~'''~~		*Return precautions: LUQ pain, lightheadedness/syncope, worsening fatigue, fevers, bleeding/bruising; avoid contact sports

	==See Also==		==See Also==

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2026-03-19T16:12:39Z

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Ostermayer: Created page with "==Background== The spleen normally weighs 150-200g in adults and is not palpable on exam A palpable spleen tip below the left costal margin generally indicates '''at least 1.5-2× enlargement''' — by the time a spleen is palpable, it is significantly enlarged *'''Massive splenomegaly''' (spleen extends beyond the umbilicus or >20 cm on imaging) has a narrower differential: chronic myeloproliferative disorders (especially CML), myelofibrosis, hairy cell leukemia, mala..."

2026-03-10T00:54:20Z

Created page with "==Background== *The spleen normally weighs 150-200g in adults and is not palpable on exam *A palpable spleen tip below the left costal margin generally indicates '''at least 1.5-2× enlargement''' — by the time a spleen is palpable, it is significantly enlarged *'''Massive splenomegaly''' (spleen extends beyond the umbilicus or >20 cm on imaging) has a narrower differential: chronic myeloproliferative disorders (especially CML), myelofibrosis, hairy cell leukemia, mala..."

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==Background==
*The spleen normally weighs 150-200g in adults and is not palpable on exam
*A palpable spleen tip below the left costal margin generally indicates '''at least 1.5-2× enlargement''' — by the time a spleen is palpable, it is significantly enlarged
*'''Massive splenomegaly''' (spleen extends beyond the umbilicus or >20 cm on imaging) has a narrower differential: chronic myeloproliferative disorders (especially CML), myelofibrosis, hairy cell leukemia, malaria, kala-azar (visceral leishmaniasis), Gaucher disease
*'''Hypersplenism''' = splenomegaly + cytopenias (from splenic sequestration and destruction of blood cells) — may see anemia, thrombocytopenia, and/or leukopenia

==Clinical Features==
===Symptoms===
*Often '''asymptomatic''' — discovered incidentally
*'''Left upper quadrant pain''' or fullness, early satiety (mass effect on stomach)
*'''Left shoulder pain''' (Kehr sign — referred diaphragmatic irritation from splenic pathology, classically splenic rupture or infarct)
*Symptoms of the underlying disease (fever, weight loss, night sweats, fatigue, bleeding, bruising)

===Physical Exam===
*Palpate from the right lower quadrant toward the left costal margin with the patient supine or in right lateral decubitus position
*A palpable spleen moves with respiration, has a medial notch, and cannot be balloted (distinguishes from left kidney)
*'''Percussion:''' Castell's sign (dullness to percussion in the lowest left intercostal space in full inspiration) — sensitivity ~60-80% for splenomegaly
*Assess for associated findings: [[lymphadenopathy]], [[hepatomegaly]], [[jaundice]], petechiae/purpura, stigmata of [[liver disease]]

===ED Complications of Splenomegaly===
*'''Splenic rupture:''' Both traumatic and atraumatic (especially [[EBV]]/mononucleosis, hematologic malignancy, malaria); presents with LUQ pain, peritoneal signs, and hemodynamic instability — '''surgical emergency'''
*'''Splenic infarction:''' Sudden LUQ pain, often pleuritic; associated with embolic sources ([[atrial fibrillation]], [[endocarditis]]), myeloproliferative disorders, sickle cell disease, and hypercoagulable states
*'''Splenic abscess:''' Fever + LUQ pain + splenomegaly; associated with [[endocarditis]], IV drug use, immunosuppression; diagnosed on CT
*'''Splenic sequestration crisis:''' Acute painful enlargement with acute anemia in [[sickle cell disease]] — more common in children; can be rapidly fatal

==Differential Diagnosis==
===Infectious===
*'''Viral:''' [[EBV]] (mononucleosis — '''most common infectious cause in young adults'''), [[CMV]], [[HIV]], viral [[hepatitis]]
*'''Bacterial:''' [[Endocarditis]], [[tuberculosis]], [[brucellosis]], [[typhoid]], splenic abscess
*'''Parasitic:''' [[Malaria]] (massive splenomegaly in endemic areas), kala-azar (visceral leishmaniasis), [[schistosomiasis]]
*'''Fungal:''' Disseminated [[histoplasmosis]], disseminated [[candidiasis]] (immunocompromised)

===Hematologic Malignancy===
*'''[[Leukemia]]:''' CML (often massive), CLL, ALL, AML, hairy cell leukemia
*'''[[Lymphoma]]:''' Hodgkin and non-Hodgkin (especially splenic marginal zone lymphoma)
*'''Myeloproliferative neoplasms:''' Polycythemia vera, essential thrombocythemia, primary myelofibrosis (often massive)
*'''Myelodysplastic syndromes'''

===Congestive (Portal Hypertension)===
*'''[[Cirrhosis]]''' — '''most common cause of splenomegaly in adults in developed countries'''
*Portal vein thrombosis
*Splenic vein thrombosis
*[[Congestive heart failure]] (severe right-sided)
*Budd-Chiari syndrome

===Hemolytic Anemias===
*[[Sickle cell disease]] (before autosplenectomy in adults; splenic sequestration in children)
*Hereditary spherocytosis
*Thalassemia major
*Autoimmune hemolytic anemia
*G6PD deficiency (during hemolytic crisis)

===Autoimmune/Inflammatory===
*'''[[Systemic lupus erythematosus]]'''
*'''[[Adult-onset Still's disease]]''' / '''[[Systemic JIA]]'''
*'''[[Rheumatoid arthritis]]''' (Felty syndrome = RA + splenomegaly + neutropenia)
*'''[[Sarcoidosis]]'''
*'''[[Macrophage activation syndrome]]''' / '''[[Hemophagocytic lymphohistiocytosis]]'''

===Infiltrative/Storage Diseases===
*Gaucher disease (most common lysosomal storage disease; can cause massive splenomegaly)
*Niemann-Pick disease
*Amyloidosis
*Glycogen storage diseases

===Other===
*Splenic cysts (congenital, post-traumatic, hydatid)
*Splenic hemangioma or other benign tumors
*Metastatic disease to spleen (rare)

===Massive Splenomegaly (Short Differential)===
''Spleen palpable >8 cm below costal margin or >20 cm on imaging:''
*CML
*Myelofibrosis
*Hairy cell leukemia
*Malaria (chronic/hyperreactive)
*Kala-azar (visceral leishmaniasis)
*Gaucher disease
*Polycythemia vera
*Splenic marginal zone lymphoma

==Evaluation==
===Workup===
*'''CBC with differential and peripheral blood smear''' — cytopenias suggest hypersplenism; blasts or abnormal cells suggest malignancy; spherocytes or schistocytes suggest hemolysis; atypical lymphocytes suggest EBV/CMV
*'''Reticulocyte count''' — elevated in hemolytic anemias
*'''[[LFTs]], albumin, INR''' — assess for liver disease/cirrhosis
*'''[[LDH]], haptoglobin, indirect bilirubin''' — hemolysis panel
*'''[[CRP]], ESR''' — inflammatory/infectious evaluation
*'''[[Ferritin]]''' — markedly elevated in MAS/HLH, AOSD/sJIA, malignancy
*'''Peripheral blood smear''' — '''do not skip this''' — may show blasts (leukemia), teardrop cells (myelofibrosis), target cells (thalassemia/liver disease), hairy cells, or parasites (malaria)
*'''[[Blood cultures]]''' — if febrile
*'''Monospot or EBV VCA IgM''' — in young adults with pharyngitis, lymphadenopathy, and splenomegaly
*'''[[HIV]]''' — screen in all unexplained splenomegaly
*'''Hepatitis B/C serologies''' — if liver disease suspected
*'''Imaging:'''
**'''[[Ultrasound]]''' — first-line to confirm and measure spleen size; also assesses liver, portal vein, and hepatic vasculature
**'''CT abdomen with contrast''' — if concern for splenic infarction, abscess, mass, or if evaluating for lymphadenopathy/malignancy
*'''[[Lactate]], coagulation studies''' — if concern for sepsis, splenic rupture, or DIC
*'''Type and screen''' — if concern for splenic rupture or significant anemia

==Management==
*'''Treat the underlying cause'''
*'''Splenic rupture:''' Hemodynamic resuscitation, emergent surgical consultation, massive transfusion protocol if indicated (see [[Splenic injury]])
*'''Splenic infarction:''' Supportive care, analgesia, anticoagulation if embolic source identified; surgical consultation if concern for abscess formation or rupture
*'''Splenic abscess:''' IV antibiotics + interventional radiology or surgical drainage
*'''Splenic sequestration crisis (sickle cell):''' Aggressive IV fluids, transfusion (target Hgb rise of 1-2 g/dL — avoid over-transfusion which can cause auto-transfusion from the spleen and hyperviscosity), emergent hematology consultation
*'''Activity restriction:''' Counsel patients with newly diagnosed splenomegaly (especially EBV/mononucleosis) to '''avoid contact sports and heavy lifting''' until splenomegaly has resolved — risk of splenic rupture

==Disposition==
*'''Admit:''' Splenic rupture, splenic infarction with hemodynamic instability, splenic abscess, splenic sequestration crisis, new diagnosis of hematologic malignancy, unexplained massive splenomegaly with cytopenias, suspected MAS/HLH
*'''Discharge with urgent follow-up (24-72h):''' Hemodynamically stable patients with incidentally discovered mild-moderate splenomegaly and non-critical initial labs; ensure follow-up with hematology, hepatology, or primary care with pending workup communicated
*'''Return precautions:''' LUQ pain, lightheadedness/syncope, worsening fatigue, fevers, bleeding/bruising; '''avoid contact sports'''

==See Also==
*[[Splenic injury]]
*[[Mononucleosis]]
*[[Cirrhosis]]
*[[Leukemia]]
*[[Lymphoma]]
*[[Sickle cell disease]]
*[[Macrophage activation syndrome]]
*[[Adult-onset Still's disease]]
*[[Fever of unknown origin]]
*[[Ferritin]]

==References==
{{reflist|2}}

[[Category:Heme/Onc]]
[[Category:GI]]