Splenomegaly: Difference between revisions
Ostermayer (talk | contribs) (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...") |
(Remove refs with incorrect PMIDs (verified against PubMed)) Tag: Manual revert |
||
| (3 intermediate revisions by the same user not shown) | |||
| Line 1: | Line 1: | ||
==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 | *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== | ==Clinical Features== | ||
===Symptoms=== | ===Symptoms=== | ||
*Often | *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) | *Symptoms of the underlying disease (fever, weight loss, night sweats, fatigue, bleeding, bruising) | ||
| Line 16: | Line 16: | ||
*Palpate from the right lower quadrant toward the left costal margin with the patient supine or in right lateral decubitus position | *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) | *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]] | *Assess for associated findings: [[lymphadenopathy]], [[hepatomegaly]], [[jaundice]], petechiae/purpura, stigmata of [[liver disease]] | ||
===ED Complications of Splenomegaly=== | ===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== | ==Differential Diagnosis== | ||
===Infectious=== | ===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=== | ===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)=== | ===Congestive (Portal Hypertension)=== | ||
* | *[[Cirrhosis]] — most common cause of splenomegaly in adults in developed countries | ||
*Portal vein thrombosis | *Portal vein thrombosis | ||
*Splenic vein thrombosis | *Splenic vein thrombosis | ||
| Line 53: | Line 53: | ||
===Autoimmune/Inflammatory=== | ===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=== | ===Infiltrative/Storage Diseases=== | ||
| Line 83: | Line 83: | ||
==Evaluation== | ==Evaluation== | ||
===Workup=== | ===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== | ==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== | ==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== | ==See Also== | ||
Latest revision as of 10:25, 22 March 2026
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