Correspondence

Clinical Problem-Solving: Lucky Lady

N Engl J Med 1993; 329:279July 22, 1993

Article

To the Editor:

With reference to the Clinical Problem-Solving case in the March 4 issue,1 I was surprised that autoimmune hemolytic anemia and neutropenia were not considered in the differential diagnosis.

A pregnant woman taking 5 mg of oral prednisone daily for stable systemic lupus erythematosus presented with severe pitting edema of both legs, a grade 2 systolic murmur, and a hemoglobin level of 6.8 g per deciliter. She was treated for kidney involvement with 300 mg of intravenous hydrocortisone a day and with azathioprine, but three weeks later pneumocystis pneumonia developed. She was treated with trimethoprim-sulfamethoxazole, and agranulocytosis developed. Bone marrow examination revealed erythroid hyperplasia. After she received “pulse” prednisolone and intravenous immune globulin, her hemoglobin level increased to 10 g per deciliter.

A hemoglobin level of 6.8 g per deciliter in a pregnant woman is abnormal2. A finding of erythroid hyperplasia on bone marrow examination makes iron deficiency or anemia of chronic disease unlikely and suggests an increase in erythroid production to compensate for hemolysis. The increase in the hemoglobin level after pulse steroid administration also suggests hemolytic anemia3. Autoimmune neutropenia in lupus also responds to high-dose steroid therapy4.

Mark Levin, M.D.
Sanford R. Nalitt Institute for Cancer and Blood Related Diseases, Staten Island, NY 10305

4 References

1. 1

   Ben-Chetrit E. Lucky lady. N Engl J Med 1993;328:636-639
   Full Text | Web of Science | Medline

2. 2

   Bentley DP. Iron metabolism and anaemia in pregnancy. Clin Haematol 1985;14:613-628
   Medline

3. 3

   Steinberg AD. Management of systemic lupus erythematosus. In: Kelley WN, Harris ED Jr, Ruddy S, Sledge CB, eds. Textbook of rheumatology. 2nd ed. Vol. 2. Philadelphia: W.B. Saunders, 1985:1107.
   

4. 4

   Richert-Boe KE. Hematologic complications of rheumatic disease. Hematol Oncol Clin North Am 1987;1:301-320
   Medline

Author/Editor Response

Dr. Ben-Chetrit replies:

To the Editor: Dr. Levin asks why we did not give primary consideration to a diagnosis of autoimmune hemolytic anemia. This diagnosis could explain the patient's anemia but not the whole constellation of symptoms and clinical findings, including the proteinuria. With regard to the bone marrow examination and the response to pulse steroids, despite receiving pulse steroids, intravenous immune globulin, and granulocyte-macrophage colony-stimulating factor, the patient had no notable improvement while she continued to receive trimethoprim-sulfamethoxazole. Six days after this drug was discontinued, the leukocyte count began to rise. The blood-count results (hemoglobin level, 10.0 g per deciliter) mentioned at the end of the case reflect the situation “by the time of discharge” -- 40 days after the pulse therapy.

E. Ben-Chetrit, M.D.
Hadassah University Hospital, 91120 Jerusalem, Israel