Project Title

Refractory/Relapsed Aplastic Anemia Responds to Anti-CD52 Antibody

Authors' Affiliations

Hakam Khazrik, Division of Medical Oncology, Department of Internal Medicine, College of Medicine ,East Tennessee State University, Johnson City ,TN Muhammad Abdur Raafey ,Department of Pathology, College of Medicine , East Tennessee State University, Johnson City ,TN. Koyamangalath Krishnan,Division of Medical Oncology, Department of Internal Medicine, College of Medicine ,East Tennessee State University,Johnson City ,TN

Location

Culp Ballroom

Start Date

4-7-2022 9:00 AM

End Date

4-7-2022 12:00 PM

Poster Number

24

Faculty Sponsor’s Department

Internal Medicine

Name of Project's Faculty Sponsor

Koyamangalath Krishnan

Classification of First Author

Post-doctoral Fellow

Competition Type

Competitive

Type

Poster Case Study Presentation

Project's Category

Other Medical

Abstract or Artist's Statement

Aplastic anemia (AA) is a rare, life-threatening disorder characterized by immune mediated loss of hematopoietic stem cells (HSC) that results in bone marrow hypoplasia, pancytopenia and its complications. Majority of AA are acquired and idiopathic. Cytotoxic T cell-mediated autoimmune destruction to the HSC is the main pathophysiological mechanism. Intrinsic defects in the hematopoietic stem cell, alterations in bone marrow microenvironment, acquired clonal abnormalities and drug/viral direct injury are other described etiologies. Improvement in molecular studies also led to better understanding of cytogenetic abnormalities, somatic mutations, telomere attrition in AA which may affect treatment response, prognosis and survival. HSCT or immunosuppression therapy (IST) can restore normal hematopoiesis. HSCT is curative, but eligibility depends on age, comorbidities, and available donors. HSCT use is limited by matched donor availability. The alternative option is IST. The standard today is intensive triple therapy with anti-thymocyte globulin (ATG), cyclosporin (CsA) and thrombopoietin receptor agonist, eltrombopag (EPAG) or with ATG and CsA in patients who require less intense IST.

72 year-old male presented with pancytopenia (WBC 1.7K/uL, Hgb 12.9 g/dL, Platelet count 6 K/uL, and ANC 600, absolute reticulocyte count 18x10*9. Bone marrow biopsy revealed markedly hypocellular marrow (

Response rates for first line IST is about 60–70%. The response and its duration are strong surrogate markers for prolonged survival. Triple therapy with hATG, CsA and EPAG improved response rate to 90% in AA in 6 months. Bone marrow evaluation in relapse setting is essential to rule out clonal evolution into MDS/AML or subtle adult inherited bone marrow syndromes. Survival is limited after second relapse in elderly. Our patient had accelerated response and CR with sustained survival after alemtuzumab, CsA and romiplostim. This report highlights a rare response to alemtuzumab and an alternate TPO mimetic, romiplostim. Given his exposure to various methods of IST and the bone marrow showing clonal evolution, he is at risk of myeloid malignancies.

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Apr 7th, 9:00 AM Apr 7th, 12:00 PM

Refractory/Relapsed Aplastic Anemia Responds to Anti-CD52 Antibody

Culp Ballroom

Aplastic anemia (AA) is a rare, life-threatening disorder characterized by immune mediated loss of hematopoietic stem cells (HSC) that results in bone marrow hypoplasia, pancytopenia and its complications. Majority of AA are acquired and idiopathic. Cytotoxic T cell-mediated autoimmune destruction to the HSC is the main pathophysiological mechanism. Intrinsic defects in the hematopoietic stem cell, alterations in bone marrow microenvironment, acquired clonal abnormalities and drug/viral direct injury are other described etiologies. Improvement in molecular studies also led to better understanding of cytogenetic abnormalities, somatic mutations, telomere attrition in AA which may affect treatment response, prognosis and survival. HSCT or immunosuppression therapy (IST) can restore normal hematopoiesis. HSCT is curative, but eligibility depends on age, comorbidities, and available donors. HSCT use is limited by matched donor availability. The alternative option is IST. The standard today is intensive triple therapy with anti-thymocyte globulin (ATG), cyclosporin (CsA) and thrombopoietin receptor agonist, eltrombopag (EPAG) or with ATG and CsA in patients who require less intense IST.

72 year-old male presented with pancytopenia (WBC 1.7K/uL, Hgb 12.9 g/dL, Platelet count 6 K/uL, and ANC 600, absolute reticulocyte count 18x10*9. Bone marrow biopsy revealed markedly hypocellular marrow (

Response rates for first line IST is about 60–70%. The response and its duration are strong surrogate markers for prolonged survival. Triple therapy with hATG, CsA and EPAG improved response rate to 90% in AA in 6 months. Bone marrow evaluation in relapse setting is essential to rule out clonal evolution into MDS/AML or subtle adult inherited bone marrow syndromes. Survival is limited after second relapse in elderly. Our patient had accelerated response and CR with sustained survival after alemtuzumab, CsA and romiplostim. This report highlights a rare response to alemtuzumab and an alternate TPO mimetic, romiplostim. Given his exposure to various methods of IST and the bone marrow showing clonal evolution, he is at risk of myeloid malignancies.