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Leukemic phase of primary cutaneous anaplastic large-cell lymphoma (ALK-negative), with downregulation of CD30

Leukemic phase of primary cutaneous anaplastic large-cell lymphoma (ALK-negative), with... Leukemic phase of anaplastic large cell lymphoma (ALCL) is extremely rare. We present a case of primary cutaneous-ALCL (C-ALCL), who developed nodal involvement 4 years later. The patient was treated with combination chemotherapy and autologous stem cell transplantation; however, she relapsed 5 months later. At that time, the patient was given brentuximab vedotin (BV) with initial response. One year later, the patient demonstrated progressive adenopathy and leukemic involvement by her ALCL (comparative T cell clonality showed PCR products of the same size in the diagnostic skin and relapsed leukemic blood). Interestingly, the circulating tumor cells showed only subset expression of CD30 by flow cytometry (performed on peripheral blood). This patient demonstrates an unusual manifestation of ALCL (leukemic phase), with the additional confounder of CD30 downregulation, perhaps secondary to the BV therapy, or possible CD30-negative tumor escape. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Hematopathology Springer Journals

Leukemic phase of primary cutaneous anaplastic large-cell lymphoma (ALK-negative), with downregulation of CD30

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Publisher
Springer Journals
Copyright
Copyright © 2018 by Springer-Verlag GmbH Germany, part of Springer Nature
Subject
Medicine & Public Health; Pathology; Hematology
ISSN
1868-9256
eISSN
1865-5785
DOI
10.1007/s12308-018-0318-2
Publisher site
See Article on Publisher Site

Abstract

Leukemic phase of anaplastic large cell lymphoma (ALCL) is extremely rare. We present a case of primary cutaneous-ALCL (C-ALCL), who developed nodal involvement 4 years later. The patient was treated with combination chemotherapy and autologous stem cell transplantation; however, she relapsed 5 months later. At that time, the patient was given brentuximab vedotin (BV) with initial response. One year later, the patient demonstrated progressive adenopathy and leukemic involvement by her ALCL (comparative T cell clonality showed PCR products of the same size in the diagnostic skin and relapsed leukemic blood). Interestingly, the circulating tumor cells showed only subset expression of CD30 by flow cytometry (performed on peripheral blood). This patient demonstrates an unusual manifestation of ALCL (leukemic phase), with the additional confounder of CD30 downregulation, perhaps secondary to the BV therapy, or possible CD30-negative tumor escape.

Journal

Journal of Hematopathology – Springer Journals

Published: Mar 2, 2018

References

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    Al-Rohil, RN; Torres-Cabal, CA; Patel, A
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