Contributions
Abstract: PB1531
Type: Publication Only
Session title: Chronic myeloid leukemia - Clinical
Background
Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML) occurring as a secondary malignancy is a rare clinical scenario, the publications on treatment-related CML (tr-CML) in lymphoma patients is limited by a few case reports.
Aims
To describe a unique case of an elderly patient who developed CML subsequent to splenic marginal zone lymphoma (SMZL). To our knowledge, CML as a secondary malignancy in a SMZL patient is hitherto unreported worldwide.
Methods
A case study.
Results
Male, 61-year-old, diagnosed with SMZL in February 2009. He had white blood cell count (WBC) of 33.1x109/L, 86% lymphocytes in peripheral blood, immunophenotype with positive for HLA-DR, CD19, CD20, cCD79α, κ, negative for CD10, CD5, BCL-2, CD103, CD23, normal karyotype and splenomegaly. The patient was asymptomatic, so a watch-and-wait approach was used. Follow-up observation showed stable disease till 2012. From Jun 2012, chlorambucil was applied intermittently, due to thrombocytopenia and progressive enlargement of the spleen. Thereafter the disease was generally stable. In Aug 2014, the patient presented with an elevated WBC of 87.98x109/L. Peripheral blood smear revealed increased immature granulocytes, with 4% promyelocyte, 12% myelocyte, 3% metamyelocyte. The BM smear identified the cellular hyperplasia and increased myeloid to erythroid ratio, with 61.5% marrow myelocytes and 31% marrow mature lymphocytes. Cytogenetic analysis revealed t (9;22) and RT-PCR analysis demonstrated BCR/ABL fusion transcript. Over six years after the initial diagnosis of SMZL, a diagnosis of CML in chronic phase was made. He started on imatinib (400mg daily), and was simultaneously treated with cyclophosphamide for the SMZL because he refused the anti-lymphoma immuno-targeting therapy. Imatinib treatment was switched to nilotinib (300mg, twice per day) after seven months due to recurrent skin rash and refractory mucous membrane ulcer, which were probably attributed to imatinib. As of Dec 2020, the patient is in sustained complete molecular response from CML, and the disease state of SMZL is stable.
Conclusion
In conclusion, we report a rare tr-CML case of an elderly patient treating with an alkylating agent for SMZL. Tr-CML is an infrequent complication after chemotherapy and/or radiotherapy, which may increase the risk of secondary malignancies by inducing genetic damage. Nilotinib, the second generation TKI, could show a favorable response in Ph+ tr-CML, similar to that in de novo CML.
Keyword(s): Chronic myeloid leukemia, Second malignancy, Splenic marginal zone lymphoma
Abstract: PB1531
Type: Publication Only
Session title: Chronic myeloid leukemia - Clinical
Background
Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML) occurring as a secondary malignancy is a rare clinical scenario, the publications on treatment-related CML (tr-CML) in lymphoma patients is limited by a few case reports.
Aims
To describe a unique case of an elderly patient who developed CML subsequent to splenic marginal zone lymphoma (SMZL). To our knowledge, CML as a secondary malignancy in a SMZL patient is hitherto unreported worldwide.
Methods
A case study.
Results
Male, 61-year-old, diagnosed with SMZL in February 2009. He had white blood cell count (WBC) of 33.1x109/L, 86% lymphocytes in peripheral blood, immunophenotype with positive for HLA-DR, CD19, CD20, cCD79α, κ, negative for CD10, CD5, BCL-2, CD103, CD23, normal karyotype and splenomegaly. The patient was asymptomatic, so a watch-and-wait approach was used. Follow-up observation showed stable disease till 2012. From Jun 2012, chlorambucil was applied intermittently, due to thrombocytopenia and progressive enlargement of the spleen. Thereafter the disease was generally stable. In Aug 2014, the patient presented with an elevated WBC of 87.98x109/L. Peripheral blood smear revealed increased immature granulocytes, with 4% promyelocyte, 12% myelocyte, 3% metamyelocyte. The BM smear identified the cellular hyperplasia and increased myeloid to erythroid ratio, with 61.5% marrow myelocytes and 31% marrow mature lymphocytes. Cytogenetic analysis revealed t (9;22) and RT-PCR analysis demonstrated BCR/ABL fusion transcript. Over six years after the initial diagnosis of SMZL, a diagnosis of CML in chronic phase was made. He started on imatinib (400mg daily), and was simultaneously treated with cyclophosphamide for the SMZL because he refused the anti-lymphoma immuno-targeting therapy. Imatinib treatment was switched to nilotinib (300mg, twice per day) after seven months due to recurrent skin rash and refractory mucous membrane ulcer, which were probably attributed to imatinib. As of Dec 2020, the patient is in sustained complete molecular response from CML, and the disease state of SMZL is stable.
Conclusion
In conclusion, we report a rare tr-CML case of an elderly patient treating with an alkylating agent for SMZL. Tr-CML is an infrequent complication after chemotherapy and/or radiotherapy, which may increase the risk of secondary malignancies by inducing genetic damage. Nilotinib, the second generation TKI, could show a favorable response in Ph+ tr-CML, similar to that in de novo CML.
Keyword(s): Chronic myeloid leukemia, Second malignancy, Splenic marginal zone lymphoma