Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow
Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin Lymphoma commonly presenting as a solid tumor either by nodal or extra-nodal manifestations. Here we describe two atypical presentations of lymphoma, finally resulting in the diagnosis of DLBCL. Case 1: A 53-year-old man with a previous...
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2022-01-01
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| Series: | Clinical Pathology |
| Online Access: | https://doi.org/10.1177/2632010X211070774 |
| _version_ | 1818964682825793536 |
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| author | Wan Awatif Wan Mohd Zohdi Ahmad Zulhimi Ismail Nurasyikin Yusof Azlin Ithnin Salwati Shuib Noraidah Masir Sivakumar Palaniappan Nor Rafeah Tumian |
| author_facet | Wan Awatif Wan Mohd Zohdi Ahmad Zulhimi Ismail Nurasyikin Yusof Azlin Ithnin Salwati Shuib Noraidah Masir Sivakumar Palaniappan Nor Rafeah Tumian |
| author_sort | Wan Awatif Wan Mohd Zohdi |
| collection | DOAJ |
| description | Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin Lymphoma commonly presenting as a solid tumor either by nodal or extra-nodal manifestations. Here we describe two atypical presentations of lymphoma, finally resulting in the diagnosis of DLBCL. Case 1: A 53-year-old man with a previous history of nasopharyngeal carcinoma presented with a two-week history of B-symptoms and hyperleukocytosis. Peripheral blood film showed 78% abnormal mononuclear cells. Immunohistochemical stain showing Ki-67 of 90%, negative c-myc, BCL2 and BCL6, and negative c-MYC with fluorescence in-situ hybridization studies on the trephine biopsy, concluded the diagnosis of CD5+ DLBCL of ABC subtype. He received intravenous cyclophosphamide and oral prednisolone for cytoreduction, followed by 6 cycles of chemo-immunotherapy. However, he succumbed due to severe sepsis after the completion of therapy. Case 2: A 56-year-old lady who was initially investigated for pyrexia of unknown origin was noted to have hemophagocytosis upon bone marrow aspirate examination. The bone marrow trephine biopsy revealed some atypical clusters of B-cells positive for CD20 which was inconclusive. PET-CT scan noted an enlarged hypermetabolic spleen without lymphadenopathy. Splenic biopsy with immunohistochemical studies revealed DLBCL of ABC subtype. The diagnosis was consistent with primary splenic DLBCL. She became unwell post splenic biopsy and was admitted to the intensive care unit where she passed away 2 weeks later from Candida and Sternotrophomonas septicemia. These cases highlight the atypical presentations of a common subtype of NHL in our center. Arriving at the definitive diagnosis can be difficult especially when patients are acutely ill, hampering the necessary invasive procedures for diagnosis. The outcomes of both cases are briefly discussed hoping to spread awareness among clinicians on the rare and acutely critical presentations of DLBCL. |
| first_indexed | 2024-12-20T13:05:00Z |
| format | Article |
| id | doaj.art-ae6b278c9ab240fa80c1416164c02940 |
| institution | Directory Open Access Journal |
| issn | 2632-010X |
| language | English |
| last_indexed | 2024-12-20T13:05:00Z |
| publishDate | 2022-01-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Clinical Pathology |
| spelling | doaj.art-ae6b278c9ab240fa80c1416164c029402022-12-21T19:39:49ZengSAGE PublishingClinical Pathology2632-010X2022-01-011510.1177/2632010X211070774Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone MarrowWan Awatif Wan Mohd Zohdi0Ahmad Zulhimi Ismail1Nurasyikin Yusof2Azlin Ithnin3Salwati Shuib4Noraidah Masir5Sivakumar Palaniappan6Nor Rafeah Tumian7Department of Medicine, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Pathology, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Pathology, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Pathology, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Pathology, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Pathology, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Kuala Lumpur, MalaysiaDiffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin Lymphoma commonly presenting as a solid tumor either by nodal or extra-nodal manifestations. Here we describe two atypical presentations of lymphoma, finally resulting in the diagnosis of DLBCL. Case 1: A 53-year-old man with a previous history of nasopharyngeal carcinoma presented with a two-week history of B-symptoms and hyperleukocytosis. Peripheral blood film showed 78% abnormal mononuclear cells. Immunohistochemical stain showing Ki-67 of 90%, negative c-myc, BCL2 and BCL6, and negative c-MYC with fluorescence in-situ hybridization studies on the trephine biopsy, concluded the diagnosis of CD5+ DLBCL of ABC subtype. He received intravenous cyclophosphamide and oral prednisolone for cytoreduction, followed by 6 cycles of chemo-immunotherapy. However, he succumbed due to severe sepsis after the completion of therapy. Case 2: A 56-year-old lady who was initially investigated for pyrexia of unknown origin was noted to have hemophagocytosis upon bone marrow aspirate examination. The bone marrow trephine biopsy revealed some atypical clusters of B-cells positive for CD20 which was inconclusive. PET-CT scan noted an enlarged hypermetabolic spleen without lymphadenopathy. Splenic biopsy with immunohistochemical studies revealed DLBCL of ABC subtype. The diagnosis was consistent with primary splenic DLBCL. She became unwell post splenic biopsy and was admitted to the intensive care unit where she passed away 2 weeks later from Candida and Sternotrophomonas septicemia. These cases highlight the atypical presentations of a common subtype of NHL in our center. Arriving at the definitive diagnosis can be difficult especially when patients are acutely ill, hampering the necessary invasive procedures for diagnosis. The outcomes of both cases are briefly discussed hoping to spread awareness among clinicians on the rare and acutely critical presentations of DLBCL.https://doi.org/10.1177/2632010X211070774 |
| spellingShingle | Wan Awatif Wan Mohd Zohdi Ahmad Zulhimi Ismail Nurasyikin Yusof Azlin Ithnin Salwati Shuib Noraidah Masir Sivakumar Palaniappan Nor Rafeah Tumian Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow Clinical Pathology |
| title | Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow |
| title_full | Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow |
| title_fullStr | Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow |
| title_full_unstemmed | Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow |
| title_short | Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow |
| title_sort | rare but potentially fatal presentations of diffuse large b cell lymphoma leukemic phase or hemophagocytic syndrome in bone marrow |
| url | https://doi.org/10.1177/2632010X211070774 |
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