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 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

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 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

The Effect of Aloe Vera Solution on Chemotherapy-Induced Stomatitis in Clients with Lymphoma and Leukemia: A Randomized Controlled Clinical Trial

Background: Stomatitis is the most common complication of chemotherapy. This study aimed to assess the effect of aloe vera solution on stomatitis and its pain intensity in patients undergoing chemotherapeutic procedures. Methods: In this randomized controlled clinical trial, 64 patients with Acute Myeloid Leukemia and Acute Lymphocytic Leukemia undergoing chemotherapy were randomly divided into a control and an intervention group. The intervention group patients were asked to wash their mouths with 5 ml of aloe vera solution for two minutes three times a day for 14 days. The control group patients, however, used only the ordinary mouthwashes recommended in hematologic centers. The patients’ mouths were examined by two assistants on days 1, 3, 5, 7, and 14. The intensity of stomatitis was recorded according to WHO stomatitis intensity checklists and pain was evaluated using Visual Analog Scale. The data were analyzed by SPSS statistical software, version 18. Results: The results showed that aloe vera solution mouthwash significantly reduced the intensity of stomatitis and its pain in the intervention group compared to the control group. On the first day, no significant difference was found between the two groups regarding the mean intensity of stomatitis (P=0.178) and pain (P=0.154). However, a significant difference was observed between the two groups in this regard on other days (days 3-14: P=0.001 for stomatitis intensity, P=0.001 for pain). Conclusions: Aloe vera solution can improve the patients’ nutritional status, reduce stomatitis and its pain intensity, and increase the patients’ satisfaction

Determination of the Prevalence of HGV Infection in Leukemia Patients

Background: The potential of acute and chronic viral hepatitis infections in blood leukocytes is one of the possible risk factors of leukemia. Between hepatitis viruses, HGV is important for unknown mechanism of pathogenesis in affecting the outcome of leukemia. In this research the prevalence of HGV infection was studied for evaluation of the possible role of this viral infection in the pathophysiology of leukemia. Methods: In this study, 100 EDTA treated blood samples were collected for 2 and half a year from different types of leukemia and also healthy control group, respectively. The plasma of these blood samples were harvested and stored at -80°C till laboratory tests were performed. Serological and antigenic markers of HBV, HCV and HGV were analyzed by ELISA methods. The HGV viremia was also studied by an in-house nested-RT-PCR method in plasma samples of patients with leukemia and control population. Results: Anti-E2-Ab was detected in 1(1%) of leukemia patients who has not the history of HBV and HCV infections. HGV-RNA was diagnosed in 4(4%) of patients with leukemia and 1(1%) of control group. Simultaneous infection of HGV-RNA and HBsAg was detected in one of patients with leukemia. Significant difference was seen between the prevalence of HBV infection in leukemia patients and control group (P=0.02). Also significant correlation was detected between sex and the prevalence of HBV infection in leukemia patients (P=0.02). None of studied risk factors of leukemia were not significantly correlated with HGV infection. Conclusion: In this study the low prevalence of active and persistent HGV infections in leukemia patients in comparing with control population was confirmed. Also detection of HGV and HBV co-infection in these patients, announced the need of completed studies in different populations with different hematological malignancies and/or abnormalities, for better therapeutic and laboratory management of these cancers

Determination of Serologic and Molecular Prevalence of Hepatitis Type B, C, and G Infections in Patients with Hematological Malignancy in the South of Fars Province, Iran

Background & Objectives: Hepatitis type G virus (HGV) is a flavivirus with RNA genome which has high nucleotide and amino acid similarity with HCV. HGV can infect blood lymphocytes for long period and may have role in inducing or complicating the clinical outcomes in patients with hematological malignancies. Therefore in this study the prevalence of HGV, HCV, and HBV infections were evaluated in patients with hematological malignancies. Materials & Methods: In this study EDTA treated blood samples were collected from 100 patients with hematological malignancies and 110 healthy controls of southern cities of Fars province (Lar, Lamerd, Grash) Iran. The serologic and molecular markers of HGV, HCV, and HBV were analyzed by ELISA and PCR and RT-PCR methods, respectively in Hematology Research Center and Transplant Research Center, Namazi hospital, Shiraz University of Medical Sciences. Also the role of some risk factors in pathogenesis of these hepatitis viruses was studied statistically. Results: Antibody against E2 antigen of HGV was diagnosed in 5% and 1.1% of patients with hematological malignancies and healthy controls, respectively. Significant difference was found between the prevalence of HGV antibodies in patients with hematological malignancies and healthy controls (P=0.037). The HCV antibody and prevalence of HCV- RNA was detected in 7% and 4% of patients with hematological malignancies respectively. Significant difference was found between the prevalence of HCV-RNA in patients with hematological malignancies and healthy controls (P=0.02). Also HBV viremia was found in 2% of patients. Conclusion: In this study the significant presentation of HGV and HCV were found in patients with hematological malignancies compared with healthy controls. However, the results suggest that similar study should be carryout to evaluate the prevalence of this viral infection in other part of Iran, to control the spreading of these infections to other people

FMS-Like Tyrosine Kinase 3 (FLT3) and Nucleophosmin 1 (NPM1) in Iranian Adult Acute Myeloid Leukemia Patients with Normal Karyotypes: Mutation Status and Clinical and Laboratory Characteristics

Objective: In this study, we evaluated the frequency of FMS-like tyrosine kinase 3 (FLT3-ITD and FLT3-TKD) and nucleophosmin (NPM1) mutations in Iranian patients with cytogenetically normal acute myeloid leukemia (CN-AML). The clinical and laboratory characteristics were compared between wild-type and mutant cases. Materials and Methods: Seventy newly diagnosed de novo AML patients were recruited at the time of diagnosis prior to chemotherapy; among them, 54 had CN-AML. For detecting mutations, the FLT3 and NPM1 genes were amplified by the polymerase chain reaction method, followed by direct sequencing. Results: Our results showed that the frequencies of FLT3-ITD, FLT3-TKD, and NPM1 mutations in CN-AML patients were 25.9%, 5.9%, and 20.8%, respectively. The most frequent NPM1 mutation type was the type A mutation. The FLT3-ITD mutation was seen more frequently in non-M3 patients compared with M3 patients. No mutation was observed in either the FLT3-TKD or the NPM1 gene in patients in the M3 French-American-British group. There was no significant association between the presence of FLT3-ITD and NPM1 mutations in CN-AML patients (p>0.05). The frequency of FLT3-ITD, FLT3-TKD, and NPM1 mutation was higher in CN-AML patients in comparison with AML patients with cytogenetic aberrations, although the differences were not statistically significant (p>0.05). There were no significant differences in mean white blood cell and platelet counts, serum hemoglobin levels, and bone marrow blast percentages between patients with wild-type and mutant FLT3-ITD and NPM1 genes (p>0.05). No difference was observed in the frequency of FLT3-ITD or NPM1 mutation regarding age or sex (p>0.05). Conclusion: Given the high stability of NPM1 during the disease course, it can be used in combination with FLT3 as well as other known genetic markers to monitor patients, especially for minimal residual disease detection

The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura

This report describes a novel mutation in the TSP1-6 domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura. See related perspective article on page 166