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

Ahmad Monabbati,Ali Adib,Seyedsajjad Eghbali,Akbar Safaei,Mani Ramzi,Sadat Noori

doi:10.1155/2016/8507317

Abstract

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.

Highlights

Sickle cell disease (SCD) is an important hereditary hemoglobinopathy which affects more than 300000 annual births worldwide mostly in low- and middle- income countries [1].Its usual clinical manifestations include anemia, hyperbilirubinemia, and vasoocclusive complications [2]
Our report describes a young male who presented with persistent multiple cervical lymphadenopathy and mild splenomegaly for about 5 years
Histomorphologic review of the resected lymph node showed well defined secondary follicles as well as several monomorphic nodules composed of monotonous population of small lymphocytes mainly with regular round nuclei, inconspicuous nucleoli, and scant cytoplasm

Summary

Introduction

Sickle cell disease (SCD) is an important hereditary hemoglobinopathy which affects more than 300000 annual births worldwide mostly in low- and middle- income countries [1]. Its usual clinical manifestations include anemia, hyperbilirubinemia, and vasoocclusive complications [2]. While peripheral blood smear shows anemia associated with presence of some sickle red blood cells (RBC), target cells, and a few nucleated RBCs, bone marrow is hyperplastic because of compensatory hyperplasia of erythroid precursors. Mild splenomegaly is seen during childhood, in early phase of disease. Thereafter, progressive shrinkage of the spleen due to continuous scarring occurs by adolescence or early adulthood, a process called autosplenectomy [4]. Premature mortality occurs due to the complications of this disease, especially cardiopulmonary organ dysfunction and chronic kidney injury [5]. This article presents a 22-year-old boy newly diagnosed with SCD who had cervical lymphadenopathy as an atypical sign

Case Presentation

Discussion