Abstract
In this report, we present a case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) associated with autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA).
A 71 year-old man was admitted to our hospital with fever, severe anemia and rapidly enlarging generalized lymphadenopathy. A diagnosis of AILD was confirmed by lymph node biopsy. Coombs test was positive and cold agglutinin titers were substantially elevated (×8192). Hemoglobin and hematocrit were markedly decreased (Hb: 4.9g/dl, Ht: 13.8%) with no reticulocyte in the peripheral blood and no erythroid precursor cells in the bone marrow. Bone marrow culture studies showed (1) decreased numbers of erythrocytic colony forming units (CFU-E) (19.8 colonies/105 cells: control 72.9) and (2) suppression of normal human CFU-E by IgG in the patient's serum (suppression rate: whole serum 40.1%, IgG 29.8%). It suggests that the positive Coombs test, the elevation of cold agglutinin titer and the presence of the IgG inhibitor erythropoiesis could be attributed to AILD, which developed AIHA and PRCA.
After 12 weeks of treatment with prednisolone (60mg/day), vincristine (1.0mg/wk), azathioprine (50mg/day) and anabolic steroid (400mg/wk), AILD was improved at first and then AIHA and PRCA, sequentially. After 3 months of the treatment, the patient developed subacute hepatitis and died from hepatic failure and pneumonia.
At autopsy, there was complete recovery from AILD and erythroid precursor cells were seen normally in the bone marrow. In addition, pneumocystis carinii bronchopneumonia and dissemination of aspergillosis were found.
A case of AILD with AIHA and PRCA has been presented with discussion of its possible pathogenesis.
