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Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge

Patient: Male, 71-year-old Final Diagnosis: Chronic lymphocytic leukemia • small lymphocytic lymphoma Symptoms: Subcutaneous indurations • subcutaneous mass Medication:— Clinical Procedure: Skin biopsy Specialty: Hematology OBJECTIVE: Unusual clinical course BACKGROUND: Most patients with chronic ly...

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Autores principales: Aldapt, Mahmood B., Soliman, Dina, Szabados, Lajos, Sharaf Eldean, Mouhammad Z., Shwaylia, Hawraa M., Abdulla, Mohammad A.J., Yassin, Mohamed A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7244225/
https://www.ncbi.nlm.nih.gov/pubmed/32388531
http://dx.doi.org/10.12659/AJCR.920411
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author Aldapt, Mahmood B.
Soliman, Dina
Szabados, Lajos
Sharaf Eldean, Mouhammad Z.
Shwaylia, Hawraa M.
Abdulla, Mohammad A.J.
Yassin, Mohamed A.
author_facet Aldapt, Mahmood B.
Soliman, Dina
Szabados, Lajos
Sharaf Eldean, Mouhammad Z.
Shwaylia, Hawraa M.
Abdulla, Mohammad A.J.
Yassin, Mohamed A.
author_sort Aldapt, Mahmood B.
collection PubMed
description Patient: Male, 71-year-old Final Diagnosis: Chronic lymphocytic leukemia • small lymphocytic lymphoma Symptoms: Subcutaneous indurations • subcutaneous mass Medication:— Clinical Procedure: Skin biopsy Specialty: Hematology OBJECTIVE: Unusual clinical course BACKGROUND: Most patients with chronic lymphocytic leukemia (CLL) are asymptomatic at diagnosis, but 10% present with B symptoms. Most patients have palpable lymphadenopathy, while 20–50% of the patients have hepatosplenomegaly. Cutaneous infiltrations in patients with CLL can be localized or generalized in the form of erythematous papules, plaques, nodules and, ulceration, which is uncommon. CASE REPORT: We present the case of a 71-year-old man diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with atypical immunophenotype and increased prolymphocytes (CLL/PLL), which was treated initially after white blood counts (WBC) doubling with Bendamustine and Rituximab for 6 cycles, and achieved complete remission. The patient relapsed after 6 months of completion of treatment, with multiple large sub-cutaneous masses, proved to be infiltration with the same atypical CLL/SLL on tissue biopsy, with pathologic features indicating disease progression. The lack of similar reported cases, and the aggressiveness of the tumor clinically and histopathologically, resulted in the decision to treat with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone (R-CHOP) as a case of aggressive lymphoma, with complete remission clinically and radiologically. CONCLUSIONS: We present a rare case of subcutaneous extramedullary masses of atypical CLL/SLL. The high proliferation index (Ki-67) and the increase of large cells are suggestive of aggressive progression of the disease; however, no frank features of Richter’s transformation were noted. Based on this and because of the unusual aggressive-looking skin masses, the panel decided to treat the patient with R-CHOP. The impact of this presentation on the prognosis of the disease is not clear. To date, our patient has responded well to treatment with R-CHOP, with complete remission of the subcutaneous masses and on PET scan, but further follow-up is needed.
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spelling pubmed-72442252020-05-29 Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge Aldapt, Mahmood B. Soliman, Dina Szabados, Lajos Sharaf Eldean, Mouhammad Z. Shwaylia, Hawraa M. Abdulla, Mohammad A.J. Yassin, Mohamed A. Am J Case Rep Articles Patient: Male, 71-year-old Final Diagnosis: Chronic lymphocytic leukemia • small lymphocytic lymphoma Symptoms: Subcutaneous indurations • subcutaneous mass Medication:— Clinical Procedure: Skin biopsy Specialty: Hematology OBJECTIVE: Unusual clinical course BACKGROUND: Most patients with chronic lymphocytic leukemia (CLL) are asymptomatic at diagnosis, but 10% present with B symptoms. Most patients have palpable lymphadenopathy, while 20–50% of the patients have hepatosplenomegaly. Cutaneous infiltrations in patients with CLL can be localized or generalized in the form of erythematous papules, plaques, nodules and, ulceration, which is uncommon. CASE REPORT: We present the case of a 71-year-old man diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with atypical immunophenotype and increased prolymphocytes (CLL/PLL), which was treated initially after white blood counts (WBC) doubling with Bendamustine and Rituximab for 6 cycles, and achieved complete remission. The patient relapsed after 6 months of completion of treatment, with multiple large sub-cutaneous masses, proved to be infiltration with the same atypical CLL/SLL on tissue biopsy, with pathologic features indicating disease progression. The lack of similar reported cases, and the aggressiveness of the tumor clinically and histopathologically, resulted in the decision to treat with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone (R-CHOP) as a case of aggressive lymphoma, with complete remission clinically and radiologically. CONCLUSIONS: We present a rare case of subcutaneous extramedullary masses of atypical CLL/SLL. The high proliferation index (Ki-67) and the increase of large cells are suggestive of aggressive progression of the disease; however, no frank features of Richter’s transformation were noted. Based on this and because of the unusual aggressive-looking skin masses, the panel decided to treat the patient with R-CHOP. The impact of this presentation on the prognosis of the disease is not clear. To date, our patient has responded well to treatment with R-CHOP, with complete remission of the subcutaneous masses and on PET scan, but further follow-up is needed. International Scientific Literature, Inc. 2020-05-10 /pmc/articles/PMC7244225/ /pubmed/32388531 http://dx.doi.org/10.12659/AJCR.920411 Text en © Am J Case Rep, 2020 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Articles
Aldapt, Mahmood B.
Soliman, Dina
Szabados, Lajos
Sharaf Eldean, Mouhammad Z.
Shwaylia, Hawraa M.
Abdulla, Mohammad A.J.
Yassin, Mohamed A.
Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge
title Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge
title_full Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge
title_fullStr Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge
title_full_unstemmed Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge
title_short Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge
title_sort subcutaneous masses as an unusual manifestation of relapse in a case of atypical chronic lymphocytic leukemia with prolymphocytoid transformation and complex karyotype: a diagnostic dilemma and treatment challenge
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7244225/
https://www.ncbi.nlm.nih.gov/pubmed/32388531
http://dx.doi.org/10.12659/AJCR.920411
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