What is massive splenomegaly?
Splenomegaly is enlargement of the spleen. However, massive splenomegaly is usually defined as a spleen extending into the left lower quadrant or pelvis or spleen which has crossed the midline of the abdomen. Massive spleen is weigh at least 500 to 1000g. Once the spleen is palpable in physical examination, it has usually reached twice its normal size.
Cause of massive splenomegaly:
1. Myeloproliferative disease:
a. Chronic myeloid leukemia
b. Polycythemia vera: clonal expansion of haematopoiesis. Lab findings include increased WBC and platelet counts.
2. Gaucher disease: autosomal recessive lyosomal glycolipid storage disorder due to insufficient acid beta-glucosidase enzyme. This leads to clinical manifestation of hepatosplenomegaly, anemia, thrombocytopenia and bone disease.
3. Lymphoma:
a. Chronic lymphocytic leukemia
b. Hairy cell leukemia: uncommon chronic B-cell lymphopoliferative disease.
c. Thalassemia major: b-Thalassemia major is a hereditary anemia resulting from decreased production of b-globin chains. Decreased b-chain synthesis results in excess free a chains that form insoluble tetramers and precipitate within the RBC, causing increased fragility and cell death.
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