SPLENOMEGALY

The spleen is frequently secondarily involved in a wide variety of systemic diseases. In virtually all instances, the response of the spleen causes its enlargement (splenomegaly), which produces a set of stereotypical signs and symptoms. Evaluation of splenomegaly is a common clinical problem that is aided considerably by knowledge of the usual limits of the splenic enlargement that is seen in the context of specific disorders. It would be erroneous to attribute enlargement of the spleen into the pelvis to vitamin B12 deficiency, or to entertain a diagnosis of CML in the absence of significant splenomegaly. As an aid to diagnosis, then, we present the following list of disorders, classified according to the degree of splenomegaly that is characteristically produced:

A. Massive splenomegaly (weight more than 1000 gm)

1. Chronic myeloproliferative disorders (chronic myeloid leukemia, myeloid metaplasia with myelofibrosis)

2. Chronic lymphocytic leukemia

3. Hairy cell leukemia

4. Lymphomas

5. Malaria

6. Gaucher disease

7. Primary tumors of the spleen (rare)

B. Moderate splenomegaly (weight 500-1000 gm)

1. Chronic congestive splenomegaly (portal hypertension or splenic vein obstruction)

2. Acute leukemias (inconstant)

3. Hereditary spherocytosis

4. Thalassemia major

5. Autoimmune hemolytic anemia

6. Amyloidosis

7. Niemann-Pick disease

8. Langerhans histiocytosis

9. Chronic splenitis (especially with infective endocarditis)

10. Tuberculosis, sarcoidosis, typhoid

11. Metastatic carcinoma or sarcoma

C. Mild splenomegaly (weight