A Unique Hairy Cell Leukemia Variant
A 65-year-old woman presented with easy bruising, left upper quadrant pain, decreased appetite, and weight loss. She had splenomegaly and lymphocytosis (lymphocyte count of 11.6 × 109/l), with remarkably abnormal appearing morphology. Her hemoglobin and platelet counts were normal. Peripheral blood...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2016-06-01
|
| Series: | Case Reports in Oncology |
| Subjects: | |
| Online Access: | http://www.karger.com/Article/FullText/446696 |
| _version_ | 1818134575656730624 |
|---|---|
| author | Charles Jian Cyrus C. Hsia |
| author_facet | Charles Jian Cyrus C. Hsia |
| author_sort | Charles Jian |
| collection | DOAJ |
| description | A 65-year-old woman presented with easy bruising, left upper quadrant pain, decreased appetite, and weight loss. She had splenomegaly and lymphocytosis (lymphocyte count of 11.6 × 109/l), with remarkably abnormal appearing morphology. Her hemoglobin and platelet counts were normal. Peripheral blood flow cytometry revealed a monoclonal B-cell population expressing CD11c, CD25, CD19, CD20, and CD103. An initial diagnosis of hairy cell leukemia (HCL) was made, and the patient was treated with a standard 5-day course of cladribine. However, her lymphocytosis improved transiently, with a relapse 4 months later. There was no improvement in her splenomegaly. An HCL variant (HCL-v) was considered based on her resistance to treatment with a purine nucleoside analog. A subsequent splenectomy improved symptoms. Two years after, the patient suffered a relapse and underwent 6 cycles of CHOP-R (cyclophosphamide, hydroxydaunomycin, oncovin, prednisone, and rituximab), achieving partial remission. While under observation, she progressed with lymphocytosis 6 months later and was treated with pentostatin. There was no significant improvement in her disease, and she died 8 weeks following treatment initiation. HCL-v is a clinically more aggressive mature B-cell lymphoma than HCL with worse splenomegaly, higher lymphocyte counts, and resistance to typical HCL therapy with purine nucleoside analogs. Early recognition of HCL-v in the history, physical examination, and investigations with morphology and flow cytometry is key to patient management. Further, as in our case of HCL-v, cell morphology can be distinctly atypical, with large nucleoli and extremely convoluted nuclei. The distinction between HCL and HCL-v is important as HCL-v patients require more aggressive therapy and closer follow-up. |
| first_indexed | 2024-12-11T09:10:48Z |
| format | Article |
| id | doaj.art-ed9a544f7bda410187efa72b404b33ee |
| institution | Directory Open Access Journal |
| issn | 1662-6575 |
| language | English |
| last_indexed | 2024-12-11T09:10:48Z |
| publishDate | 2016-06-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Oncology |
| spelling | doaj.art-ed9a544f7bda410187efa72b404b33ee2022-12-22T01:13:30ZengKarger PublishersCase Reports in Oncology1662-65752016-06-019231231610.1159/000446696446696A Unique Hairy Cell Leukemia VariantCharles JianCyrus C. HsiaA 65-year-old woman presented with easy bruising, left upper quadrant pain, decreased appetite, and weight loss. She had splenomegaly and lymphocytosis (lymphocyte count of 11.6 × 109/l), with remarkably abnormal appearing morphology. Her hemoglobin and platelet counts were normal. Peripheral blood flow cytometry revealed a monoclonal B-cell population expressing CD11c, CD25, CD19, CD20, and CD103. An initial diagnosis of hairy cell leukemia (HCL) was made, and the patient was treated with a standard 5-day course of cladribine. However, her lymphocytosis improved transiently, with a relapse 4 months later. There was no improvement in her splenomegaly. An HCL variant (HCL-v) was considered based on her resistance to treatment with a purine nucleoside analog. A subsequent splenectomy improved symptoms. Two years after, the patient suffered a relapse and underwent 6 cycles of CHOP-R (cyclophosphamide, hydroxydaunomycin, oncovin, prednisone, and rituximab), achieving partial remission. While under observation, she progressed with lymphocytosis 6 months later and was treated with pentostatin. There was no significant improvement in her disease, and she died 8 weeks following treatment initiation. HCL-v is a clinically more aggressive mature B-cell lymphoma than HCL with worse splenomegaly, higher lymphocyte counts, and resistance to typical HCL therapy with purine nucleoside analogs. Early recognition of HCL-v in the history, physical examination, and investigations with morphology and flow cytometry is key to patient management. Further, as in our case of HCL-v, cell morphology can be distinctly atypical, with large nucleoli and extremely convoluted nuclei. The distinction between HCL and HCL-v is important as HCL-v patients require more aggressive therapy and closer follow-up.http://www.karger.com/Article/FullText/446696B-cell lymphoid leukemiaHairy cell leukemia variant |
| spellingShingle | Charles Jian Cyrus C. Hsia A Unique Hairy Cell Leukemia Variant Case Reports in Oncology B-cell lymphoid leukemia Hairy cell leukemia variant |
| title | A Unique Hairy Cell Leukemia Variant |
| title_full | A Unique Hairy Cell Leukemia Variant |
| title_fullStr | A Unique Hairy Cell Leukemia Variant |
| title_full_unstemmed | A Unique Hairy Cell Leukemia Variant |
| title_short | A Unique Hairy Cell Leukemia Variant |
| title_sort | unique hairy cell leukemia variant |
| topic | B-cell lymphoid leukemia Hairy cell leukemia variant |
| url | http://www.karger.com/Article/FullText/446696 |
| work_keys_str_mv | AT charlesjian auniquehairycellleukemiavariant AT cyruschsia auniquehairycellleukemiavariant AT charlesjian uniquehairycellleukemiavariant AT cyruschsia uniquehairycellleukemiavariant |