A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings
Hemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a comp...
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Wiley
2012-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2012/326053 |
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| author | Ing S. Tiong Michael B. Y. Lau Seventeen Toumoua Shingirai Chiruka |
| author_facet | Ing S. Tiong Michael B. Y. Lau Seventeen Toumoua Shingirai Chiruka |
| author_sort | Ing S. Tiong |
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| description | Hemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a complete response. Twenty-four days after the last chemotherapy, he presented with febrile neutropaenia and was diagnosed with HLH. The diagnosis was based upon persistent fever, pancytopenia, hyperferritinemia, splenomegaly, and hemophagocytosis on bone marrow aspirate. He began treatment with dexamethasone, cyclosporine, and etoposide. Fever resolved and hyperferritinemia improved but pancytopenia persisted. He died 13 days later from septic shock with positive blood cultures. A limited postmortem examination was performed and biopsies were taken from bone marrow, liver, and spleen. Biopsies demonstrated abundant hemophagocytosis by the activated macrophage as stained by CD68. There was no evidence of residual CLL as demonstrated by the lack of lymphocytes which was confirmed by the negative staining of CD79a. Chemotherapy appears to be responsible for the development of HLH in this patient. This is the second reported case of HLH developing after a rituximab-containing chemotherapy. |
| format | Article |
| id | doaj-art-c6a74af13aea448586c380a5532c7215 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-c6a74af13aea448586c380a5532c72152025-02-03T05:44:40ZengWileyCase Reports in Hematology2090-65602090-65792012-01-01201210.1155/2012/326053326053A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy FindingsIng S. Tiong0Michael B. Y. Lau1Seventeen Toumoua2Shingirai Chiruka3Southern Blood and Cancer Service, Dunedin Hospital, Private Bag 1921, Dunedin 9016, New ZealandDivision of Haematology, Southern Community Laboratories Ltd., Dunedin 9016, New ZealandDivision of Haematology, Southern Community Laboratories Ltd., Dunedin 9016, New ZealandSouthern Blood and Cancer Service, Dunedin Hospital, Private Bag 1921, Dunedin 9016, New ZealandHemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a complete response. Twenty-four days after the last chemotherapy, he presented with febrile neutropaenia and was diagnosed with HLH. The diagnosis was based upon persistent fever, pancytopenia, hyperferritinemia, splenomegaly, and hemophagocytosis on bone marrow aspirate. He began treatment with dexamethasone, cyclosporine, and etoposide. Fever resolved and hyperferritinemia improved but pancytopenia persisted. He died 13 days later from septic shock with positive blood cultures. A limited postmortem examination was performed and biopsies were taken from bone marrow, liver, and spleen. Biopsies demonstrated abundant hemophagocytosis by the activated macrophage as stained by CD68. There was no evidence of residual CLL as demonstrated by the lack of lymphocytes which was confirmed by the negative staining of CD79a. Chemotherapy appears to be responsible for the development of HLH in this patient. This is the second reported case of HLH developing after a rituximab-containing chemotherapy.http://dx.doi.org/10.1155/2012/326053 |
| spellingShingle | Ing S. Tiong Michael B. Y. Lau Seventeen Toumoua Shingirai Chiruka A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings Case Reports in Hematology |
| title | A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings |
| title_full | A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings |
| title_fullStr | A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings |
| title_full_unstemmed | A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings |
| title_short | A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings |
| title_sort | case of hemophagocytic lymphohistiocytosis in a patient with chronic lymphocytic leukemia after treatment with fludarabine cyclophosphamide and rituximab chemotherapy with autopsy findings |
| url | http://dx.doi.org/10.1155/2012/326053 |
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