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LETTER

Reversible Marked Splenomegaly in Pernicious Anemia

right arrow Rajiv K. Pruthi; Ayalew Tefferi; and Robert M. Petitt

15 November 1993 | Volume 119 Issue 10 | Page 1053


TO THE EDITOR:

Pernicious anemia is characterized by macrocytosis, neuropathy, and mucosal atrophy of the gastrointestinal tract, but marked splenomegaly is not considered to be a characteristic. We describe a patient with a marked enlargement of the spleen that resolved after vitamin B12 therapy.

In September 1991, a 46-year-old white man presented with fatigue. Physical findings included pallor and splenomegaly palpable at 8 cm below the left costal margin. Laboratory findings showed pancytopenia and macrocytosis. The patient had a leukocyte count of 2.0 x 109, with a normal differential; a hemoglobin concentration of 57 g/L; a mean corpuscular volume of 119 fL; a platelet count of 141 x 109/L; a total bilirubin of 2.1 mg/dL, with a normal direct fraction (other liver function tests were normal); and a lactic dehydrogenase level of 2940 U/L. A peripheral blood smear contained oval macrocytes and hypersegmented neutrophils, and the patient's bone marrow showed characteristic megaloblastic changes. The diagnosis was confirmed with an undetectable vitamin B12, normal serum folate, positive intrinsic factor antibody, and Schilling test. The splenomegaly and pancytopenia resolved 3 months after initiation of vitamin B12 therapy.

In the first half of the century, the reported incidence of splenomegaly in pernicious anemia varied from 3% to 45% [1-3], and the size ranged from 3 to 14 cm below the left costal margin [3]. Palpable splenomegaly in patients with pernicious anemia reportedly declined after the introduction of "liver therapy" in 1926 [1, 4]. Subsequently, Bigg [2] reported an incidence of 3% (6 of 200 patients). Of the 6 patients, 2 had no other condition that could account for the observed splenomegaly. In one of these patients, the spleen size regressed with treatment.

Thus, splenomegaly is probably a consistent feature of protracted, untreated pernicious anemia, which is now rare due to earlier recognition and institution of specific replacement therapy. Unlike in a previously reported case [5], the splenomegaly seen in our patient was totally reversed with vitamin B12 therapy. We conclude therefore that although marked splenomegaly may occur in pernicious anemia, it is both rare and reversible with treatment.


References
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1. Davidson S. Clinical picture of pernicious anemia prior to introduction of liver therapy in 1926 and in Edinburgh subsequent to 1944. BMJ. 1957; 2:241-3.

2. Bigg E. Spleen size in pernicious anemia. Ann Intern Med. 1940; 14: 277-80.

3. MacCarty WC. Chronic splenomegaly. Arch Intern Med. 1928; 41: 536-48.

4. Minot GR, Murphy WP. Treatment of pernicious anemia by a special diet. JAMA. 1926. 87:470-6.

5. Enokohara H, Saito K, Hazama S, Hirose K, Komatsu H, Furusawa S. A case of pernicious anemia with giant splenomegaly. Japanese Journal of Clinical Hematology. 1978; 19:1684-9.

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