Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease

Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed...

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Main Authors: Ahmad Monabbati, Sadat Noori, Akbar Safaei, Mani Ramzi, Seyedsajjad Eghbali, Ali Adib
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2016/8507317
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author Ahmad Monabbati
Sadat Noori
Akbar Safaei
Mani Ramzi
Seyedsajjad Eghbali
Ali Adib
author_facet Ahmad Monabbati
Sadat Noori
Akbar Safaei
Mani Ramzi
Seyedsajjad Eghbali
Ali Adib
author_sort Ahmad Monabbati
collection DOAJ
description Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules. To rule out possibility of a malignant process involving lymph nodes, an immunohistochemical panel was ordered which was in favor of benign mantle cell hyperplasia. Immunoglobulin gene rearrangement study showed no clonal bands and confirmed benign nature of the process. Respecting mild abnormalities on Complete Blood Count, peripheral blood smear was reviewed revealing some typical sickle red blood cells as well as rare nucleated red blood cells. Solubility test for hemoglobin (HB) S was positive. Hemoglobin electrophoresis confirmed diagnosis of homozygous HbS disease.
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series Case Reports in Hematology
spelling doaj-art-9bb15acca5cd4c4fa03128184d7e01f52025-08-20T03:36:54ZengWileyCase Reports in Hematology2090-65602090-65792016-01-01201610.1155/2016/85073178507317Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell DiseaseAhmad Monabbati0Sadat Noori1Akbar Safaei2Mani Ramzi3Seyedsajjad Eghbali4Ali Adib5Department of Pathology, Shiraz University of Medical Sciences, Shiraz, IranDepartment of Pathology, Shiraz University of Medical Sciences, Shiraz, IranDepartment of Pathology, Shiraz University of Medical Sciences, Shiraz, IranDepartment of Hematology-Oncology and Stem Cell Transplantation, Shiraz University of Medical Sciences, Shiraz, IranDepartment of Pathology, Bushehr University of Medical Sciences, Bushehr, IranStudent Research Committee, Shiraz University of Medical Sciences, Shiraz, IranSickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules. To rule out possibility of a malignant process involving lymph nodes, an immunohistochemical panel was ordered which was in favor of benign mantle cell hyperplasia. Immunoglobulin gene rearrangement study showed no clonal bands and confirmed benign nature of the process. Respecting mild abnormalities on Complete Blood Count, peripheral blood smear was reviewed revealing some typical sickle red blood cells as well as rare nucleated red blood cells. Solubility test for hemoglobin (HB) S was positive. Hemoglobin electrophoresis confirmed diagnosis of homozygous HbS disease.http://dx.doi.org/10.1155/2016/8507317
spellingShingle Ahmad Monabbati
Sadat Noori
Akbar Safaei
Mani Ramzi
Seyedsajjad Eghbali
Ali Adib
Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease
Case Reports in Hematology
title Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease
title_full Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease
title_fullStr Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease
title_full_unstemmed Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease
title_short Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease
title_sort mantle cell hyperplasia of peripheral lymph nodes as initial manifestation of sickle cell disease
url http://dx.doi.org/10.1155/2016/8507317
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