Small B-cell neoplasm responding to ibrutinib after 17 years of cold agglutinin disease symptom

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  • 17年にわたる寒冷凝集素症の経過後に顕在化しibrutinibが有効であった小型B細胞腫瘍
  • 17年にわたる寒冷凝集素症の経過後に顕在化しibrutinibが有効であった小型B細胞腫瘍 : 第114回近畿血液学地方会 優秀演題
  • 17ネン ニ ワタル カンレイ ギョウシュウソショウ ノ ケイカ ゴ ニ ケンザイカ シ ibrutinib ガ ユウコウ デ アッタ コガタ Bサイボウ シュヨウ : ダイ114カイ キンキ ケツエキガク チホウカイ ユウシュウ エンダイ

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Abstract

<p>In this study, we report a case of a 77-year-old woman who was presented with anemia in the winter of 2002. She was diagnosed with cold agglutinin disease (CAD) and treated with corticosteroids. Further, her hemoglobin levels were maintained between 7.0 g/dl and 8.0 g/dl. In May 2019, mature peripheral blood lymphocytes increased with exacerbation of hemolytic anemia. The lymphocytes were positive for CD19 and CD20, but negative for CD5, CD10, and CD23. Additionally, they were positive for cell surface IgM-κ. The B-cell neoplasm could not be further subclassified due to the lack of BCL2-IgH and BCL1-IgH rearrangement and morphology. The IgM-κ-type M-protein was found in serum, and the direct Coombs test was negative for IgG but positive for C3b/C3d. These findings suggested that small B-cell neoplasm-associated M-protein was involved in the development of CAD through complement activation. Based on the presence of TP53 deletion, the patient was treated with ibrutinib monotherapy. Although hemolysis rapidly improved with a dramatic decrease in lymphocytes, she died from a cerebral hemorrhage. It is assumed that ibrutinib improved CAD through suppression of small B-cell neoplasm-related M-protein. CAD can precede lymphoproliferative disorders; however, the risk of ibrutinib-associated hemorrhage should be noted.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 62 (12), 1678-1683, 2021

    The Japanese Society of Hematology

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