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Yamaguchi K, Yamamoto G, Watanabe O, Kageyama K, Kaji D, Taya Y, Nishida A, Ishiwata K, Takagi S, Yamamoto H, Asano-Mori Y, Uruga H, Ito S, Takazawa Y, Wake A, Uchida N, Taniguchi S. Discordant lymphoma characterized by the coexistence of diffuse large B-cell lymphoma in the brain and mantle cell lymphoma in the colon, rectum, and bone marrow. Brain Tumor Pathol 2025; 42:26-32. [PMID: 40100513 DOI: 10.1007/s10014-025-00499-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2024] [Accepted: 03/06/2025] [Indexed: 03/20/2025]
Abstract
We describe a rare case of discordant lymphoma characterized by the coexistence of diffuse large B-cell lymphoma (DLBCL) in the brain and mantle cell lymphoma (MCL) in the colon, rectum, and bone marrow. A 63-year-old male patient with consciousness impairment and gait disturbance was admitted to our institution. Head computed tomography scan and contrast-enhanced magnetic resonance imaging showed a mass in the right temporal lobe and rectal wall thickening. Brain biopsy revealed DLBCL, and bone marrow and rectum biopsy showed MCL. According to a polymerase chain reaction analysis of immunoglobulin heavy-chain gene rearrangements using brain and bone marrow specimens, the two lesions were clonally unrelated lymphomas. After five cycles of R-MPV (rituximab, methotrexate, procarbazine, vincristine) therapy and three cycles of R-ESHAP (rituximab, etoposide, cytarabine, cisplatin, methylprednisolone) therapy, the patient received autologous hematopoietic stem cell transplantation using R-MEAM (rituximab, ranimustine, etoposide, cytarabine, melphalan) regimen after bridging therapy with ibrutinib. In addition, he received whole-brain irradiation at a dose of 40 Gy in 20 fractions as consolidation therapy. He did not relapse within 3 years of transplantation. To the best of our knowledge, this is the first case report of DLBCL and MCL coexistence.
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Affiliation(s)
| | - Go Yamamoto
- Department of Hematology, Toranomon Hospital, Tokyo, Japan
| | - Otoya Watanabe
- Department of Hematology, Toranomon Hospital, Tokyo, Japan
| | - Kosei Kageyama
- Department of Hematology, Toranomon Hospital, Tokyo, Japan
| | - Daisuke Kaji
- Department of Hematology, Toranomon Hospital, Tokyo, Japan
| | - Yuki Taya
- Department of Hematology, Toranomon Hospital, Tokyo, Japan
| | - Aya Nishida
- Department of Hematology, Toranomon Hospital Kajigaya, Kanagawa, Japan
| | - Kazuya Ishiwata
- Department of Hematology, Toranomon Hospital Kajigaya, Kanagawa, Japan
| | | | | | | | - Hironori Uruga
- Department of Pathology, Toranomon Hospital, Tokyo, Japan
| | - Shinji Ito
- Department of Pathology, Toranomon Hospital, Tokyo, Japan
| | | | - Atsushi Wake
- Department of Hematology, Toranomon Hospital Kajigaya, Kanagawa, Japan
| | - Naoyuki Uchida
- Department of Hematology, Toranomon Hospital, Tokyo, Japan
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Tashakori M, Thakral B, Tang G, Khoury JD, Medeiros LJ. From the archives of MD Anderson Cancer Center: A case of concurrent follicular lymphoma and Langerhans cell sarcoma with a review of the literature. Ann Diagn Pathol 2021; 52:151720. [PMID: 33676171 DOI: 10.1016/j.anndiagpath.2021.151720] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2021] [Revised: 01/26/2021] [Accepted: 02/08/2021] [Indexed: 10/22/2022]
Abstract
Transdifferentiation of follicular lymphoma to a Langerhans cell neoplasm is rarely reported and not well understood. Here we present a case, review the literature and discuss some of the biological underpinnings of lineage switch of B cells to histiocytes/Langerhans cells. A 31-year-old woman had follicular lymphoma (FL) and Langerhans cell sarcoma (LCS) co-localized above and below diaphragm. The FL was low-grade, had typical morphologic features, and was positive for CD10, BCL-2, and BCL-6. The LCS was cytologically atypical with necrosis and a high mitotic rate, and the immunophenotype supported Langerhans cell lineage positive for CD1a, CD207/langerin, and S-100 protein. Both tumors carried IGH-BCL2 and the LCS cells had immunophenotypic evidence of a residual B cell program, supporting the notion that these neoplasms are clonally related. The case reported is unusual because the patient was young and both diseases presented simultaneously, before any therapy. In addition, immunohistochemical analysis showed that the LCS was negative for BRAF V600E and phospho-ERK, suggesting that the LCS belongs to the known subset of Langerhans cell tumors lacking BRAF V600E and MAP2K1 mutations. Concurrent occurrence of FL and Langerhans cell neoplasm is an unusual phenomenon, with 10 cases reported previously: 4 Langerhans cell histiocytosis and 6 Langerhans cell sarcoma, including this case.
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Affiliation(s)
- Mehrnoosh Tashakori
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America
| | - Beenu Thakral
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America
| | - Guilin Tang
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America
| | - Joseph D Khoury
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America
| | - L Jeffrey Medeiros
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America.
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3
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Synchronous discordant Epstein-Barr virus (EBV)–positive nodal T/NK-cell lymphoma and EBV-positive diffuse large B cell lymphoma in a patient with a history of EBV-positive Burkitt lymphoma. J Hematop 2018. [DOI: 10.1007/s12308-018-0334-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
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Langerhans cell histiocytosis associated with lymphoma: an incidental finding that is not associated with BRAF or MAP2K1 mutations. Mod Pathol 2017; 30:734-744. [PMID: 28084334 PMCID: PMC5839484 DOI: 10.1038/modpathol.2016.235] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2016] [Revised: 12/02/2016] [Accepted: 12/02/2016] [Indexed: 01/24/2023]
Abstract
Langerhans cell histiocytosis is characterized by a localized or systemic proliferation of Langerhans cells. BRAF mutations have been reported in 40-70% of cases and MAP2K1 mutations have been found in BRAF-negative cases, supporting that Langerhans cell histiocytosis is a true neoplasm, at least in mutated cases. In a small subset of patients, Langerhans cell histiocytosis is detected incidentally in a biopsy involved by lymphoma. These lesions are usually minute and rarely have been assessed for mutations. We assessed for BRAF and MAP2K1 mutations in seven cases of Langerhans cell histiocytosis detected incidentally in biopsies involved by lymphoma. We performed immunohistochemical analysis for phosphorylated (p)-ERK. There were four men and three women (median age, 54 years; range, 28-84). The biopsies included lymph nodes (n=6) and chest wall (n=1). The lymphomas included five classical Hodgkin lymphoma, one mantle cell lymphoma, and one angioimmunoblastic T-cell lymphoma. All cases were negative for BRAF V600E and MAP2K1 mutations. Nevertheless, three of seven cases showed ERK activation as shown by expression of p-ERK. We performed mutation analysis using a panel of 134 commonly mutated genes (including BRAF and MAP2K1) by next-generation sequencing on three cases, including two cases positive for p-ERK by immunohistochemistry. No mutations were detected in any of the three cases assessed. Six patients received therapy appropriate for their lymphoma. With a median follow-up of 21 months (range, 6-89), no patients developed disseminated or recurrent Langerhans cell histiocytosis. We conclude that lymphoma-associated Langerhans cell histiocytosis is a clinically benign process that is not associated with BRAF V600E or MAP2K1 mutations and, as suggested by others, the designation Langerhans cell hyperplasia may be more appropriate. Nevertheless, the expression of p-ERK in three cases suggests that the RAS-RAF-MAP2K-ERK pathway is activated, perhaps by non-mutational mechanisms induced by the presence of lymphoma or lymphoma-microenvironment interactions.
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Li X, Deng QI, Li YM. A case of Langerhans' cell histiocytosis following Hodgkin's disease. Mol Clin Oncol 2016; 5:27-30. [PMID: 27330759 PMCID: PMC4906978 DOI: 10.3892/mco.2016.860] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2015] [Accepted: 02/25/2016] [Indexed: 11/20/2022] Open
Abstract
Langerhans' cell histiocytosis (LCH) is a group of disorders in various tissues characterized by the proliferation of Langerhans cells. It is rarely observed in adults. Langerhans cells are dendritic cells that express cluster of differentiation 1a (CD1a) and S100 protein, and contain Birbeck granules. Its etiopathogenesis remains to be elucidated. One possible etiological cause is a reactive proliferation of Langerhans cells following chemotherapy or radiotherapy for Hodgkin's disease (HD). A number of cases of LCH associated with malignant lymphoma have been reported previously. It may follow after the malignant lymphoma, or occur with it. However, fewer cases have been reported where the LCH followed after HD. In the present case report, a patient was diagnosed with HD following chemotherapy for LCH. As LCH was diagnosed, the patient was treated with a combination of various chemotherapeutic agents in two cycles of cyclophosphamide, vincristine, and prednisolone (COP), and eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). The patient went into a successful clinical remission. One year later, computed tomographic (CT) scans of the thorax and abdomen revealed augmentation of the tumor mass in the mediastinum. An excisional biopsy of the right inguinal lymph node was performed. The patient was diagnosed with nodular sclerosing Hodgkin's disease. Following four cycles of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (ABVD) chemotherapy, a whole-body positron emission tomographic CT scan revealed a decrease in tumor mass in the mediastinum. At present, the patient remains in treatment, and the prognosis has yet to be fully determined.
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Affiliation(s)
- Xin Li
- Department of Hematology, The First Central Hospital of Tianjin, Tianjin 300192, P.R. China
| | - Q I Deng
- Department of Hematology, The First Central Hospital of Tianjin, Tianjin 300192, P.R. China
| | - Yu-Ming Li
- Department of Hematology, The First Central Hospital of Tianjin, Tianjin 300192, P.R. China
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Zhang C, Yi Y, Chen C, Wang J, Liu Z. Discordant lymphoma consisting of mediastinal large B-cell lymphoma and nodular sclerosis Hodgkin lymphoma in the right supraclavicular lymph nodes: a case report. Diagn Pathol 2015; 10:215. [PMID: 26715530 PMCID: PMC4693434 DOI: 10.1186/s13000-015-0450-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2015] [Accepted: 12/17/2015] [Indexed: 02/12/2023] Open
Abstract
Background Discordant lymphoma is defined by the simultaneous presence of two or more distinct types of lymphomas at different anatomic sites. With fewer than 20 studies reporting cases of discordant lymphoma to date, the incidence of this condition is believed to be very low. Case Presentation Here, we report a case of discordant lymphoma in a 34-year-old female patient that involved mediastinal large B-cell lymphoma and nodular sclerosis Hodgkin lymphoma in the right supraclavicular lymph nodes. The patient presented with a mass in the mediastinum and enlargement of the right supraclavicular lymph nodes, but no obvious signs of lymphoma. Histological examination revealed that the encapsulated mediastinal mass contained medium- or large-size tumor cells with lightly stained cytoplasm and round vesicular nuclei as well as a high percentage of mitotic cells; strongly positive immunohistochemical staining for PAX5, CD20, and CD79a also was observed. Examination of biopsied right supraclavicular lymph node tissues revealed separation by collagen fibers, extensive inflammatory cell infiltration, and large-size tumor cells, such as Reed–Sternberg cells. These tissues stained strongly positive for PAX5 and CD30, weakly positive for CD15, and negative for Epstein-Barr viral RNA. We also found monoclonal gene rearrangement in the immunoglobulin heavy chain gene in the mediastinal large B-cell lymphoma, but no monoclonal gene rearrangement in the nodular sclerosis Hodgkin lymphoma. These findings suggested that these two lymphomas were not of a common clonal origin. The patient was treated by surgical excision of the mediastinal mass followed by radio-chemotherapy, and no metastasis or recurrence occurred during a follow-up period of 32 months. Conclusion A review of previously reported cases indicated that the clinical manifestations and pathological features of discordant lymphoma are diverse due to variation in the types of lymphomas involved. Physicians must have an awareness of discordant lymphoma to avoid incorrect and missed diagnoses, especially considering that the true incidence may not be as low as previously believed. Electronic supplementary material The online version of this article (doi:10.1186/s13000-015-0450-6) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Chun Zhang
- Department of Pathology, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
| | - Yuanxue Yi
- Department of Pathology, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
| | - Chunyan Chen
- Department of Pathology, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
| | - Jianrong Wang
- Department of Pathology, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
| | - Zhu Liu
- Department of Orthopaedics, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
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Topal AA, Malkani RH, Bhat G, Vishnani RT. Recalcitrant Pruritus as Primary Manifestation of Synchronous Hodgkins Lymphoma and Langerhans Cell Histiocytosis. ACTA MEDICA (HRADEC KRÁLOVÉ) 2015; 55:104-6. [DOI: 10.14712/18059694.2015.65] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
We present a 73 year old female with intractable pruritus and nonspecific cutaneous rash for a period of 9 months. She had recieved symptomatic therapy with no improvement. A complete examination revealed axillary and abdominal lymphadenopathy. A biopsy confirmed the diagnosis of Hodgkins lymphoma with Langerhans cell histiocytosis. She received 5 cycles of chemotherapy with resolution of pruritus and reduction in axillary and abdominal lymphadenopathy. The patient presented 6 months later with relapse and succumbed to the illness. Simultaneous occurrence of Langerhans cell histiocytosis and Hodgkins lymphoma may lead to misdiagnosis. The awareness of such an association is important to make an accurate diagnosis and guide appropriate therapy.
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Langerhans cell histiocytosis followed by hodgkin lymphoma: a case report. IRANIAN JOURNAL OF MEDICAL SCIENCES 2015; 40:282-6. [PMID: 25999631 PMCID: PMC4430893] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/09/2013] [Revised: 09/18/2013] [Accepted: 10/13/2013] [Indexed: 11/30/2022]
Abstract
Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells. The major pathological features of LCH are expression of CD1a and S100 as well as Birbeck granules. Its presentation can differ from a mild bone lesion to a multi-systemic evolved malignant neoplasm; however, the latter outcome is almost rare. Thus, LCH is mostly known as a benign neoplasm. In this study, we present a case of LCH followed by Hodgkin lymphoma (HL). Accompaniment of this disease with malignant lymphoma is rare and considered as case report. Several cases in which malignant lymphoma occurred prior to LCH are reported; however, few cases can be found with LCH followed by malignant lymphomas.
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Imashuku S, Shimazaki C, Tojo A, Imamura T, Morimoto A. Management of adult Langerhans cell histiocytosis based on the characteristic clinical features. World J Hematol 2013; 2:89-98. [DOI: 10.5315/wjh.v2.i3.89] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2013] [Revised: 05/10/2013] [Accepted: 06/06/2013] [Indexed: 02/05/2023] Open
Abstract
To find out the most appropriate management, clinical features of 18 cases of adult multisystem langerhans cell histiocytosis (LCH) have been analyzed. The patients comprising of 9 males and 9 females were median age of 36 years, ranging from 18-53 years at diagnosis. Regarding the initial symptoms, 7 patients (2 males and 5 females) showed central diabetes insipidus (CDI) and other endocrine symptoms with thickened pituitary stalk or a mass at the hypothalamic region. Additional 2 patients initiated the disease with CDI with no immediate diagnosis. In the remaining patients, the disease begun with single (n = 3) or multiple (n = 1) spinal bone lesion(s) in 4 patients (all males), with multiple bone lesions in 3 patients (1 male and 2 females), with single skull lesion in one female patient and with ambiguous symptoms including hypothyroidism in the remaining one male patient. We also recognized the correlation between pregnancy/childbirth and LCH in 4 patients. In terms of treatment, 9 patients received systemic immuno-chemotherapy alone, of which the majority received vinblastine-based chemotherapy while 4 received 2-chlorodeoxyadenosine. Five had a combination of immuno-chemotherapy with surgical resection or radiotherapy, 2 had immunotherapy alone, 2 had surgical resection followed by observation alone to date. Three patients received hematopoietic stem cell transplantation after extensive chemotherapy. In terms of outcome, 15 patients are alive (9 with active disease, 6 without active disease), with a median of 66 mo (range 17-166 mo), two died of disease while the remaining 1 lost to follow-up. Based on these results, we think that early diagnosis and rapid introduction of appropriate treatment are essential, in order to overcome the problems relevant to adult LCH.
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Park IKS, Park IK, Kim EK, Kim S, Jeon SR, Huh JR, Suh CW. Langerhans cell histiocytosis followed by Hodgkin's lymphoma. Korean J Intern Med 2012; 27:459-62. [PMID: 23269889 PMCID: PMC3529247 DOI: 10.3904/kjim.2012.27.4.459] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/29/2008] [Revised: 05/21/2008] [Accepted: 06/13/2008] [Indexed: 11/27/2022] Open
Abstract
A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma.
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Affiliation(s)
- IK Soo Park
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - In Keun Park
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Eun Kyoung Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Sang Ryong Jeon
- Department of Neurosurgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Joo Ryung Huh
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Cheol Won Suh
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Llamas-Velasco M, Cannata J, Dominguez I, García-Noblejas A, Aragües M, Fraga J, Arranz R. Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver. J Cutan Pathol 2012; 39:1125-30. [DOI: 10.1111/cup.12013] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2012] [Revised: 05/20/2012] [Accepted: 06/29/2012] [Indexed: 12/25/2022]
Affiliation(s)
- Mar Llamas-Velasco
- Department of Dermatology; Hospital Universitario de La Princesa; Madrid; Spain
| | - Jimena Cannata
- Department of Haematology; Hospital Universitario de La Princesa; Madrid; Spain
| | | | - Ana García-Noblejas
- Department of Haematology; Hospital Universitario de La Princesa; Madrid; Spain
| | - Maximiliano Aragües
- Department of Dermatology; Hospital Universitario de La Princesa; Madrid; Spain
| | - Javier Fraga
- Department of Pathology; Hospital Universitario de La Princesa; Madrid; Spain
| | - Reyes Arranz
- Department of Haematology; Hospital Universitario de La Princesa; Madrid; Spain
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Benharroch D, Guterman G, Levy I, Shaco-Levy R. High content of Langerhans cells in malignant lymphoma--incidence and significance. Virchows Arch 2010; 457:63-7. [PMID: 20473767 DOI: 10.1007/s00428-010-0931-7] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2010] [Revised: 04/25/2010] [Accepted: 04/26/2010] [Indexed: 11/30/2022]
Abstract
We describe 25 patients, 14 with classical Hodgkin lymphoma and 11 with non-Hodgkin lymphoma, in all of whom an excess of Langerhans cells was evident. Except for the first three cases, in which the excess of Langerhans cells was identified on routine slides, the remaining cases were disclosed by actively investigating lymphomas with excess of CD68+ histiocytes and performing CD1a and S-100 protein immunostains. Although no clonality study was performed on the Langerhans cells, we endorse the view which states that in the above association, the Langerhans cells are polyclonal. Fourteen cases of Hodgkin lymphoma with a large number of Langerhans cells were identified in a cohort of 231 classical Hodgkin lymphomas. We compared the features of classical Hodgkin lymphoma with abundant Langerhans cells with those without Langerhans cells. Our analysis reveals that Hodgkin lymphoma with Langerhans cell excess shows greater LMP1/EBV expression (P = .007) and lower p53 expression (P = .042) in the Hodgkin-Reed-Sternberg cells but is not associated with a poorer outcome.
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Affiliation(s)
- Daniel Benharroch
- Department of Pathology, Soroka University Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.
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Dehkordi NR, Rajabi P, Naimi A, Heidarpour M. Langerhans cell histiocytosis following Hodgkin lymphoma: a case report from Iran. JOURNAL OF RESEARCH IN MEDICAL SCIENCES : THE OFFICIAL JOURNAL OF ISFAHAN UNIVERSITY OF MEDICAL SCIENCES 2010; 15:58-61. [PMID: 21526060 PMCID: PMC3082786] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/05/2009] [Accepted: 09/12/2009] [Indexed: 12/04/2022]
Abstract
The occurrence of Langerhans Cell Histiocytosis (LCH) in a patient with lymphoma is an indication of a probable relationship between them. The two conditions have similarities both clinically and histopathologically. Occurrence of these two conditions in the same patient, particularly not simultaneously, is rare. According to different management and treatment of these conditions, exact histopathologic evaluation and even using immunohistochemistery (IHC) can prevent misdiagnosis. In this report, a 10 year old boy presented who afflicted with LCH 3 years after diagnosis and treatment of mixed cellularity Hodgkin lymphoma.
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Affiliation(s)
- Nahid Reisi Dehkordi
- Assistant Professor of Pediatrics, Department of Pediatrics, Faculty of Medicine, Isfahan University of Medical Science, Isfahan, Iran
| | - Parvin Rajabi
- Professor of Pathology, Department of Pathology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
| | - Azar Naimi
- Assistant Professor of Pediatrics, Department of Pediatrics, Faculty of Medicine, Isfahan University of Medical Science, Isfahan, Iran,Pathologist, Department of Pathology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran,*Corresponding Author E-mail:
| | - Mitra Heidarpour
- Assistant Professor of Pathology, Department of Pathology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
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Kim M, Kang HJ, Shin HY, Ahn HS, Lee DS. Concurrent langerhans cell histiocytosis and B-lineage lymphoid proliferation in the bone marrow. Korean J Lab Med 2009; 29:402-5. [PMID: 19893348 DOI: 10.3343/kjlm.2009.29.5.402] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
We present three cases of concurrent Langerhans cell histiocytosis (LCH) and B-lineage lymphoid cell infiltrations and/or nodules in the bone marrow. The first patient was a 25-month-old boy who presented with LCH on the right shoulder and multiple osteolytic lesions. Bone marrow biopsy showed the presence of LCH and two large lymphoid nodules of B-lineage, which were located in the paratrabecular region. Both LCH and the lymphoid nodules resolved after treatment with prednisone, vinblastine, methotrexate, and cyclophosphamide. The second patient was a 7-month-old girl who presented with LCH in the scalp and bone marrow. In spite of the treatment, a follow-up bone marrow analysis performed after 16 months showed LCH and increased B-lineage lymphoid cells in the interstitial area. The third patient was a 26-month-old girl, and imaging studies revealed reddish skin lesions and multiple osteolytic lesions. Skin biopsy and bone marrow biopsy did not show the presence of LCH; however, we initiated the treatment on the basis of the results of imaging studies. The follow-up study after 6 months showed the presence of LCH and large, patchy infiltration of B-lymphoid cells. We report three rare cases of concurrent bone marrow involvement of LCH and B-lineage lymphoid proliferation, which strongly suggest lymphoid malignancy. Further, clonal changes should be studied to elucidate the common pathogenic mechanism between the two diseases.
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Affiliation(s)
- Miyoung Kim
- Department of Laboratory Medicine, Seoul National University College of Medicine, Seoul, Korea
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Clonally related follicular lymphomas and histiocytic/dendritic cell sarcomas: evidence for transdifferentiation of the follicular lymphoma clone. Blood 2008; 111:5433-9. [PMID: 18272816 DOI: 10.1182/blood-2007-11-124792] [Citation(s) in RCA: 235] [Impact Index Per Article: 13.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
Rare cases of histiocytic and dendritic cell (H/DC) neoplasms have been reported in patients with follicular lymphoma (FL), but the biologic relationship between the 2 neoplasms is unknown. We studied 8 patients with both FL and H/DC neoplasms using immunohistochemistry, fluorescence in situ hybridization (FISH) for t(14;18), and polymerase chain reaction (PCR)/sequencing of BCL2 and IGH rearrangements. There were 5 men and 3 women (median age, 59 years). All cases of FL were positive for t(14;18). The H/DC tumors included 7 histiocytic sarcomas, 5 of which showed evidence of dendritic differentiation, and 1 interdigitating cell sarcoma. Five H/DC tumors were metachronous, following FL by 2 months to 12 years; tumors were synchronous in 3. All 8 H/DC tumors showed presence of the t(14;18) either by FISH, or in 2 cases by PCR with the major breakpoint region (MBR) probe. PCR and sequencing identified identical IGH gene rearrangements or BCL2 gene breakpoints in all patients tested. All H/DC tumors lacked PAX5, and up-regulation of CEBPbeta and PU.1 was seen in all cases tested. These results provide evidence for a common clonal origin of FL and H/DC neoplasms when occurring in the same patient, and suggest that lineage plasticity may occur in mature lymphoid neoplasms.
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Christie LJ, Evans AT, Bray SE, Smith MEF, Kernohan NM, Levison DA, Goodlad JR. Lesions resembling Langerhans cell histiocytosis in association with other lymphoproliferative disorders: a reactive or neoplastic phenomenon? Hum Pathol 2006; 37:32-9. [PMID: 16360413 DOI: 10.1016/j.humpath.2005.08.024] [Citation(s) in RCA: 73] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/24/2005] [Revised: 08/26/2005] [Accepted: 08/30/2005] [Indexed: 11/26/2022]
Abstract
Langerhans cell histiocytosis (LCH) has been described in association with a variety of neoplasms preceding, after, or synchronous with the other tumor. In some cases, a neoplasm may arise as a complication of therapy for LCH, and in others, the association may be coincidental. Synchronous occurrence has been reported most commonly in association with malignant lymphoma in which discrete proliferations of Langerhans cells (LCs) histologically indistinguishable from LCH are seen. In most cases, these LCs are closely related to or intermingling with the primary pathology. The nature of LCs in this context remains elusive with debate as to whether they represent a true clonal neoplasm or an exaggerated reactive phenomenon. The lack of evidence for LCH progression or disease elsewhere strongly supports the latter. We have encountered 5 examples of LCH-like proliferations occurring in the context of other lymphoproliferative disorders. These include 2 cases of mycosis fungoides and 1 of cutaneous B-cell pseudolymphoma, associations that to our knowledge have not been described before. Two patients were female, and the clonality of the LC proliferation was assessed using laser capture microdissection and the human androgen receptor. The results showed that the LCs forming discrete nodules in a case of cutaneous B-cell pseudolymphoma and a case of Hodgkin's lymphoma were polyclonal. This suggests that, at least in a proportion of cases, the aggregates of LCs occasionally identified within other lymphoproliferative lesions represent a reactive proliferation rather than a potentially aggressive second neoplasm.
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MESH Headings
- Adult
- B-Lymphocytes/pathology
- Biomarkers, Tumor/metabolism
- Cell Proliferation
- Clone Cells
- Fatal Outcome
- Female
- Histiocytosis, Langerhans-Cell/complications
- Histiocytosis, Langerhans-Cell/metabolism
- Histiocytosis, Langerhans-Cell/pathology
- Humans
- Langerhans Cells/metabolism
- Langerhans Cells/pathology
- Lymphoproliferative Disorders/complications
- Lymphoproliferative Disorders/metabolism
- Lymphoproliferative Disorders/pathology
- Male
- Middle Aged
- Mycosis Fungoides/complications
- Mycosis Fungoides/pathology
- Neoplasms, Multiple Primary/complications
- Neoplasms, Multiple Primary/metabolism
- Neoplasms, Multiple Primary/pathology
- Neoplasms, Second Primary/complications
- Neoplasms, Second Primary/metabolism
- Neoplasms, Second Primary/pathology
- Pseudolymphoma/complications
- Pseudolymphoma/pathology
- Skin Neoplasms/complications
- Skin Neoplasms/pathology
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Affiliation(s)
- Lesley J Christie
- Department of Pathology, Ninewells Hospital and Medical School, Dundee DD1 9SY, UK.
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