What happens in Leukemias, Lymphomas, and Myelomas?
Tariq I Mughal, John M Goldman, Sabena T Mughal in Understanding Leukemias, Lymphomas, and Myelomas, 2017
The leukemias are a group of disorders characterized by the excessive accumulation of abnormal white cells in the bone marrow and peripheral blood. The lymphomas are a heterogeneous group of cancers that originate in lymphoid cells in lymph nodes or other lymphoid tissue. The common feature of these tumors was that the component cells all seemed to be derived from the lymphatic system. Lymphomas, in particular Non-Hodgkin lymphoma, in contrast to leukemia are considerably more common worldwide. Multiple myeloma is a cancer which arises from the plasma cells in the bone marrow and is characterized by the production of a single species of immunoglobulin molecule. The different subtypes of myeloma were not recognized until the 1930s, when electrophoresis was discovered. In tandem with the leukemias and lymphomas, much of the molecular understanding of myeloma has been achieved. Amongst the leukemias, perhaps up to 5% may arise from inherited abnormal genes.
Diagnosis of Leukemia, Lymphoma, and Myeloma
Tariq I Mughal, John M Goldman, Sabena T Mughal in Understanding Leukemias, Lymphomas, and Myelomas, 2017
This chapter discusses the clinical aspects, including the presentations and how diagnoses are made for leukemias, lymphomas, and myelomas. Patients with leukemia often present with signs and symptoms arising from bone marrow failure and organ infiltration by the leukemia cells. The marrow of patients with acute leukemia is densely packed with cells, most of them blast cells. In chronic lymphocytic leukemia, the cells normally present in the marrow are replaced to varying degrees by small lymphocytes similar to those found in excess in the blood. The majority of patients have asymmetric painless enlargement of lymph nodes in one or more peripheral lymph node regions, such as neck and axillary or groin. The diagnosis of all lymphomas is typically made by histological examination of an excised lymph node. Most patients with myeloma present with bone pain and symptoms of anemia. Undoubtedly a revised staging system should follow soon, particularly as more sophisticated analysis of myeloma molecular biology is applied.
Specific Therapy for Lymphomas
Tariq I Mughal, John M Goldman, Sabena T Mughal in Understanding Leukemias, Lymphomas, and Myelomas, 2017
Prior to the use of immunotherapy in conjunction with chemotherapy, most patients with follicular lymphoma exhibited a disease course which appeared to be unaltered by various treatments. Based on the notion that the majority of the lymphoma cells in patients with follicular lymphoma express the CD20 antigen, the monoclonal antibody rituximab has been extensively studied. Conventional lymphoma staging procedures are used to stage patients with Mantle Cell Lymphoma, but there is a paucity of information of the precise role of PET scan. In accordance to the WHO classifications, both lymphomas and lymphoid leukemias are included because both solid and circulating phases are present in many lymphoid neoplasms and the distinction between them is artificial. Testicular lymphomas are usually treated with R-cyclophosphamide, daunorubicin, vincristine, and prednisone chemo-therapy, followed by radiotherapy to the contralateral testis and prophylactic intrathecal chemotherapy. Specialists consider it appropriate to monitor these patients carefully and consider pre-emptive therapy with appropriate anti-viral drugs.
Blastic Variant of Mantle Cell Lymphoma Following Interfollicular Hodgkin's Lymphoma
Published in Leukemia & Lymphoma, 2001
Leonard E. Grosso, Brian T. Collins, John Visconti
Infrequently, patients are diagnosed with Hodgkin's lymphoma and a morphologically distinct lymphoma. While specific subtypes of lymphomas (including Hodgkin's lymphoma) may present diagnostic difficulties, fine needle aspiration biopsy (FNAB) is sometimes useful in the evaluation and classification of these lymphoproliferative processes. We report a case of the blastic variant of mantle cell lymphoma following Hodgkin's lymphoma, interfollicular variant. A 66-year-old woman with a history of Hodgkin's lymphoma presented with increasing contralateral cervical adenopathy three years after receiving chemotherapy. FNAB with ancillary immunophenotypic characterization identified mantle cell lymphoma, blastic variant. Subsequent excisional biopsy confirmed this diagnosis and also aided in the exclusion of recurrent Hodgkin's lymphoma. In addition to identifying the previously unreported combination of blastic variant of mantle cell lymphoma and Hodgkin's lymphoma, this case emphasizes the utility of FNAB in evaluation of new masses in patient's with a previous diagnosis of Hodgkin's lymphoma.
Lymphoma in acquired generalized lipodystrophy
Published in Leukemia & Lymphoma, 2016
Rebecca J. Brown, Jean L. Chan, Elaine S. Jaffe, Elaine Cochran, Alex M. DePaoli, Jean-Francois Gautier, Cecile Goujard, Corinne Vigouroux, Phillip Gorden
Acquired generalized lipodystrophy (AGL) is a rare disease thought to result from autoimmune destruction of adipose tissue. Peripheral T-cell lymphoma (PTCL) has been reported in two AGL patients. We report five additional cases of lymphoma in AGL, and analyze the role of underlying autoimmunity and recombinant human leptin (metreleptin) replacement in lymphoma development. Three patients developed lymphoma during metreleptin treatment (two PTCL and one ALK-positive anaplastic large cell lymphoma), and two developed lymphomas (mycosis fungoides and Burkitt lymphoma) without metreleptin. AGL is associated with high risk for lymphoma, especially PTCL. Autoimmunity likely contributes to this risk. Lymphoma developed with or without metreleptin, suggesting metreleptin does not directly cause lymphoma development; a theoretical role of metreleptin in lymphoma progression remains possible. For most patients with AGL and severe metabolic complications, the proven benefits of metreleptin on metabolic disease will likely outweigh theoretical risks of metreleptin in lymphoma development or progression.
Diagnosis and management of rare gastrointestinal lymphomas
Published in Leukemia & Lymphoma, 2012
Eliza A. Hawkes, Andrew Wotherspoon, David Cunningham
Primary gastrointestinal (GI) lymphoma is rare, however accounts for 30–40% of cases of extranodal lymphoma. Several lymphoma subtypes have a propensity for GI tract involvement. Whilst the literature is dominated by data related to the more common extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) and diffuse large B-cell lymphoma (DLBCL) of the stomach, this review focuses on the rare subtypes of enteropathy-associated T-cell lymphoma (EATL), GI follicular lymphoma, mantle cell lymphoma (lymphomatous polyposis coli) and extranodal natural killer (NK)/T-cell lymphoma nasal-type (ENKTL). Due to its rarity, the majority of data regarding primary GI lymphoma have been derived from subgroups of larger cohorts. Clinical characteristics, prognosis and management can differ from those of nodal disease, despite corresponding histology. We discuss these differences and the challenges associated with diagnosis and management of these rare diseases.
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