Hereditary spherocytosis | Hemolytic anemia, recurrent transfusions, intractable leg ulcers | Improves or eliminates anemia |
Hereditary elliptocytosis | Limited role for splenectomy | — |
Pyruvate kinase deficiency | Only in severe cases, recurrent transfusions | Decreased transfusion requirement, palliative only |
Glucose-6-phosphate dehydrogenase deficiency | None | — |
Warm-antibody autoimmune hemolytic anemia | Failure of medical (steroid) therapy | 60%–80% response rate, recurrences common |
Sickle cell disease | History of acute sequestration crisis, splenic symptoms, or infarction (consider concomitant cholecystectomy) | Palliative, variable response |
Thalassemia | Excessive transfusion requirements, symptomatic splenomegaly, or infarction | Diminished transfusion requirements, relief of symptoms |
Acute myeloid leukemia (AML) | Intolerable symptomatic splenomegaly | Relief of abdominal pain and early satiety |
Chronic myeloid leukemia | Symptomatic splenomegaly | Relief of abdominal pain and early satiety |
Chronic myelomonocytic leukemia | Symptomatic splenomegaly | Relief of abdominal pain and early satiety |
Essential thrombocythemia | Only for advanced disease (ie, transformation to myeloid metaplasia or AML) with severe symptomatic splenomegaly | Relief of abdominal pain and early satiety |
Polycythemia vera | Only for advanced disease (ie, transformation to myeloid metaplasia or AML) with severe symptomatic splenomegaly | Relief of abdominal pain and early satiety |
Myelofibrosis (agnogenic myeloid metaplasia) | Severe symptomatic splenomegaly | 76% clinical response at 1 year, high risk of hemorrhagic, thrombotic, and infectious complications (26%) |
Chronic lymphocytic leukemia | Cytopenias and anemia | 75% response rate |
Hairy cell leukemia | Cytopenias and symptomatic splenomegaly | 40%–70% response rate |
Hodgkin's disease | Surgical staging in select cases | — |
Non-Hodgkin's lymphoma | Cytopenias, symptomatic splenomegaly | Improved complete blood count values, relief of symptoms |
ITP | Failure of medical therapy, recurrent disease | 75%–85% rate of long-term response |
TTP | Excessive plasma exchange requirement | Typically curative |
Abscesses of the spleen | Therapy of choice | Curative |
Symptomatic parasitic cysts | Therapy of choice | Curative; exercise caution not to spill cyst contents |
Symptomatic nonparasitic cysts | Partial splenectomy for small cysts; unroofing for large cysts | Curative |
Gaucher disease | Hypersplenism | Improves cytopenias; does not correct underlying disease |
Niemann-Pick disease | Symptomatic splenomegaly | Improves symptoms; does not correct underlying disease |
Amyloidosis | Symptomatic splenomegaly | Improves symptoms; does not correct underlying disease |
Sarcoidosis | Hypersplenism or symptomatic splenomegaly | Improves symptoms and cytopenias; does not correct underlying disease |
Felty's syndrome | Neutropenia | 80% durable response rate |
Splenic artery aneurysm | Splenectomy best for distal lesions near splenic hilum | Curative |
Portal hypertension | Portal or sinistral hypertension due to splenic vein thrombosis | Palliative |