Variability of the erythropoietic response in autoimmune hemolytic anemia: analysis of 109 cases

Abstract

One hundred-nine cases of autoimmune hemolysis were reviewed to determine the frequency of reticulocytopenia, the state of the erythroid marrow in reticulocytopenic cases, and the course of reticulocyte production indices with time and glucocorticoid treatment. The mean hematocrit at presentation was 24 mL/dL, but 30% of cases had an initial hematocrit less than 20 mL/dL. Median reticulocyte percentage at diagnosis was 9%, and median reticulocyte production index was 2.8 times basal. Twenty percent of cases had an initial reticulocyte count less than 4%, and 37% had an initial reticulocyte production index less than 2.0 times basal. These reticulocytopenic patients were nearly evenly distributed between warm and cold antibody- mediated cases and between primary and secondary cases. Fifty-four percent of reticulocytopenic cases had a bone marrow examination during hospitalization. Three-fourths of these marrows showed erythroid hyperplasia, and erythroid hypoplasia was seen in only one case. Eighty- eight cases had serial reticulocyte measurements, and in only 15% of patients did the reticulocyte production index remain less than 2.0 times basal. Thus, in most cases, the initially low reticulocyte production index may represent a lag in marrow responsiveness to hemolytic stress. In cases with persistent reticulocytopenia, ineffective erythropoiesis is suggested by the frequency of marrow erythroid hyperplasia. In the cases that were initially reticulocytopenic and demonstrated an increase in reticulocyte production index, the magnitude of this increase was significantly greater in glucocorticoid-treated patients than in those not so treated, indicating that a glucocorticoid sensitive component exists in the marrow erythropoietic response to hemolysis. Awareness of the frequency of an initial reticulocytopenia in cases of autoimmune hemolysis may be important in initial diagnosis and treatment.

A Case Report of Refractory Warm Autoimmune Hemolytic Anemia Treated With Plasmapheresis and Rituximab

“…Our patient had severe disease, manifested by the rapid development of anemia in less than 96 h, minimal reticulocytosis, hemoglobinuria and a poor response to conventional therapy. The lack of reticulocytes in the peripheral smear was presumably due to the overwhelming autoantibody titer that reacted with the late orthochromic normoblasts and led to their subsequent recognition and removal by bone marrow macrophages (15). The evidence for this lies in the improved reticulocytosis observed immediately following TPE.…”

Autoimmune hemolytic anemia

“…The laboratory evaluation shows a hemoglobin and hematocrit that varies widely, from normal in the compensating patient with indolent hemolysis to severely decreased in the patient with fulminant hemolysis. The MCV usually is elevated re¯ecting reticulocytosis, but reticulocytopenia can exist early in the disorder or secondary to autoimmune hemolysis of the reticulocytes or an inadequate bone marrow response [36]. The WBC count typically exhibits a mild leukocytosis representing predominantly neutrophils, but neutropenia may also be seen.…”

Successful treatment of tacrolimus‐related pure red cell aplasia and autoimmune hemolytic anemia with rituximab in a pediatric cardiac transplant patient

“…Due to the rarity of this combined presentation, clinicians may fail to consider PRCA as a possible differential diagnosis in AIHA patients having reticulocytopenia, especially as this can be seen in association with normoblastic hyperplasia in 37% of cases of AIHA. 9,10 Reticulocytopenia in these patients is thought to reflect a concomitant immune-mediated destruction of RBC precursors, a temporary infection-induced suppression of hematopoiesis, or a delayed bone marrow response to hemolysis. 9 These differ from the typical findings on bone marrow evaluation in PRCA, which are complete or near-complete absence of erythroid precursors.…”