A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia Associated with Autoimmune Hemolytic Anemia and Pure Red Cell Aplasia

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  • 寒冷凝集素症，自己免疫性溶血性貧血及び純赤芽球ろうを合併したＡｎｇｉｏｉｍｍｕｎｏｂｌａｓｔｉｃ　Ｌｙｍｐｈａｄｅｎｏｐａｔｈｙ　ｗｉｔｈ　Ｄｙｓｐｒｏｔｅｉｎｅｍｉａの１例　　特にＡＩＨＡ・ＰＲＣＡとＡＩＬＤの病因的相関について
  • 寒冷凝集素症,自己免疫性溶血性貧血及び純赤芽球癆を合併したAngioimmunoblastic Lymphadenopathy with Dysproteinemiaの1例--特にAIHA・PRCAとAILDの病因的相関について
  • カンレイ ギョウシュウソショウ ジコ メンエキセイ ヨウケツセイ ヒンケツ オ
  • —特にAIHA·PRCAとAILDの病因的相関について—

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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).<br>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.<br>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.<br>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.<br>A case of AILD with AIHA and PRCA has been presented with discussion of its possible pathogenesis.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 22 (11), 1751-1758, 1981

    The Japanese Society of Hematology

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