Post-splenectomy response in adult patients with immune thrombocytopenic purpura

Splenectomy has been the conventional surgical treatment for patients with Immune Thrombocytopenic Purpura (ITP). Aim: To define response to surgical therapy, pre operative factors influencing outcome and tolerability of surgery in adult patients undergoing splenectomy for ITP. Method: We analyzed prospectively maintained data of 33 patients who were diagnosed as ITP and underwent splenectomy over the last 10 years. The age, presenting complaints, bleeding manifestations, clinical details and other investigations were noted. Details of immediate pre-operative administration of blood transfusions, platelet transfusions and other forms of therapy were also recorded. Operative details with regards to blood loss and the presence of accessory spleens were obtained. Postoperative course in terms of clinical improvement, rates of complications and platelet counts was also noted. Results: Skin petechiae and menorhhagia were common presenting symptoms in patients (mean age 26.5±10.5 yrs) with ITP. Eighteen patients underwent splenectomy for failure of therapy and fifteen for relapse on medical treatment. Mean platelet bags transfused in immediate pre-operative period were 2.8±0.8. Mean intra-operative blood loss was 205±70.5 ml. Accessory spleens were removed in 1 case (3.03%). The immediate postoperative response was complete in 19 cases (57.58%) and partial in 13 cases (39.39%). The platelet counts increased significantly from 23142±12680/ µL (Microliter) (mean ± SD) preoperatively to 170000±66000/µL (Microliter) within 24-48 hours after splenectomy ( P < 0.05). The mean platelet count was 165000+66000/µL (Microliter) at the end of one month when steroids were tapered off gradually. Four patients (12.12%) had complications (one each of wound hematoma, wound infection, splenic fossa collection and upper GI hemorrhage) in postoperative period but all responded to therapy. One relapsed patient was detected with accessory spleen and responded after re-surgery. Response to splenectomy was better in young patients and in those patients who had higher immediate post-splenectomy thrombocytosis. Conclusions: Splenectomy is safe and effective therapy in ITP patients with no response to steroids and relapse after medical therapy. Response to splenectomy was more in young patients and in those patients who had higher immediate post-splenectomy thrombocytosis

Wiskott-Aldrich Syndrome Presenting with JMML-Like Blood Picture and Normal Sized Platelets

Objective. The aim of this paper is to report the case of Wiskott-Aldrich syndrome (WAS) that presented with unusual laboratory features. Clinical Presentation and Intervention. Male neonate admitted with symptoms related to thrombocytopenia, whose initial diagnosis was considered as neonatal alloimmune thrombocytopenia and JMML (juvenile myelomonocytic leukemia) but subsequently diagnosis was confirmed as WAS. Conclusion. This case shows that a suspicion of WAS is warranted in the setting of neonatal thrombocytopenia with JMML-like blood picture and normal sized platelets

The Fc Receptor Polymorphisms and Expression of Neutrophil Activation Markers in Patients with Sickle Cell Disease from Western India

Objective. Sickle cell disease has variable clinical manifestations. Activation of neutrophils plays an important role in the initiation and propagation of vaso occlusive crises which can be analysed by determining the expression of neutrophil antigens such as CD16, CD32, and CD62L. The common FcγR polymorphisms (FcγRIIA and FcγRIIIB) are considered to influence clinical presentation. This study focuses on distribution of FcγR polymorphisms and their association with neutrophil activity among the patients from western India. Methods. In this paper 127 sickle cell anemia patients and 58 patients with sickle-β-thalassemia (median age 12±8.58 years) with variable clinical phenotypes along with 175 normals were investigated. FcγRs polymorphisms were analysed by RFLP and AS-PCR. Activation of neutrophils was measured by flow cytometry. Results. The genotypic frequency of the H/R genotype of FcγRIIA and the NA1/NA1 genotype of FcγRIIIB was significantly decreased in patients compared to normals (P-0.0074, P-0.0471, resp.). We found a significant difference in the expression of CD32 and CD62L among the patients as against normals. A significantly higher expression of CD32 was seen in the milder patients with the H/H genotype (P-0.0231), whereas the expression of CD16 was higher in severe patients with the NA2/NA2 genotype (P-0.0312). Conclusion. The two FcγR polymorphisms had significant association with variable phenotypes of sickle cell disease. The expression of CD62L decreased in our patients indicating activation of neutrophils

FLT3 and NPM-1 mutations in a cohort of acute promyelocytic leukemia patients from India

Background: Acute promyelocytic leukemia (APL) with t (15;17) is a distinct category of acute myeloid leukemia (AML) and is reported to show better response to anthracyclin based chemotherapy. A favorable overall prognosis over other subtypes of AML has been reported for APL patients but still about 15% patients relapse. Methods: This study evaluated the presence of Famus like tyrosine kinase-3 (FLT3) and nucleophosmin-1 (NPM1) gene mutations in a cohort of 40 APL patients. Bone marrow/peripheral blood samples from patients at the time of diagnosis and follow-up were processed for immunophenotyping, cytogenetic markers and isolation of DNA and RNA. Samples were screened for the presence of mutations in FLT3 and NPM1 genes using polymerase chain reaction followed by sequencing. Results: Frequency of FLT3/internal tandem duplication and FLT3/tyrosine kinase domain was found to be 25% and 7% respectively. We observed a high frequency of NPM1 mutation (45%) in the present population of APL patients