Indian Journal of Hematology and Blood Transfusion

Sarcoidosis and Crohns disease have been associated with increased long term risk of lymphoproliferative disorders, including lymphomas. Newly developed lymphadenopathy in a patient with these disorders should prompt pathological evaluation. Castleman’s disease is a lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with regressed follicles surrounded by expanded mantle zones of small lymphocytes, and interfollicular vascular proliferation in the hyaline-vascular type. Similar to sarcoidosis and Crohns disease, its etiology is incompletely understood, although immune dysregulation, genetic factors and infectious and environmental factors are thought to play a role in all three diseases. Interleukin-6 is a possible pathological common factor between these three disease processed. Unicentric, hyaline-vascular type Castleman’s disease can be treated successfully with complete surgical resection. We report a patient with long history of sarcoidosis and Crohns disease with newly developed lymphadenopathy which was found to be due to Castleman’s disease.

Expecting a family is an important component and a great goal for better quality of life for most of adults with β-thalassemia major. The aim of the present study was to examine the marital status of adults with β-thalassemia major. This cross-sectional study examined the marital status of patients with transfusion-dependent β-thalassemia aged over 15 years. Patients’ demographic characteristics including age, gender, marital status, duration of marriage, divorce, having or not having children and spouse’s health status were recorded. Information about the disease including cardiac and endocrine complications, ferritin level, splenectomy and viral hepatitis were also recorded. Of 228 patients with transfusion-dependent β-thalassemia major aged over 15 years who were treated at this medical center, 32 (14 %) were married. The mean age of married patients was 25.18 ± 4.74 years. Among the married patients, 8 (25 %) were females and 24 (75 %) patients were males. The mean age of marriage was 22.76 ± 4.16 years. The minimum and maximum marriage age was 15 and 33 years, respectively. The median duration of marriage was one year with the range from 3 months to 11 years. Only 8 (25 %) patients (one female and seven males) had children. Therapeutic advances have led to significantly increased survival and improved quality of life and fertility of patients with β-thalassemia major. According to the results, 14 % of patients over 15 years were married which was slightly higher as compared with other similar studies.

Mycoplasma pneumonia (M. pneumonia) is usually not considered among the several pathogens that induce immune thrombocytopenia (ITP). We report a child with a clinical diagnosis of severe ITP that was associated with M. pneumonia pneumonia, and review the few cases described in the English literature. We suggest that thrombocytopenia associated with M. pneumonia infection may constitute a subset of ITP, although unlike ITP it occurs concomitantly with the infection and tends to be more severe than “classic” ITP. We recommend that prompt specific antibiotic and immune modulating treatment should be initiated in appropriate clinical settings.

Thalassemia major can affect physical, psycho-social and economic life of patients and their parents. The aim of the study was to assess the disease knowledge and general self-efficacy among adolescents with Thalassemia major and their parents’ perspective with a view to develop an informational booklet on thalassemia. In a cross-sectional study 50 adolescents along with 48 parents were recruited from the hematology day care centre of a tertiary care facility. Ethical clearance from institute ethics committee, written informed consent from parent and assent from the adolescent were taken. Consecutive participants those who consented were enrolled in the study. Adolescents between the age group of 10–18 years, and their parents completed the self-developed subject data sheet, knowledge questionnaire and standardized general self-efficacy tools. The mean knowledge scores of the adolescent and their parents related to thalassemia were 13.9 ± 2.9 and 13.7 ± 3.6 respectively, while the perceived GSE scores of the adolescent along with the parents’ perspective were 30.1 ± 6.1 and 29.3 ± 6.5. Positive correlation between the adolescents’ knowledge with GSE (r = 0.464, p = 0.007) was observed. The knowledge of the adolescents and their parents showed significant association with the education (p = 0.001, p = 0.002) residence (p = 0.04 and p = 0.01) and parents’ employment (p = 0.04). The knowledge of adolescents with thalassemia and their parents is limited. There is need to counsel the adolescents and their parents about the disease and its management in order to improve the GSE of these adolescents.

Whole blood is composed of both cellular and plasma components, providing a rich source of therapeutic products. Of late, platelet derived biomaterial (platelet rich plasma) consisting of plasma proteins and platelets are increasingly being used for various indications. Protocols for preparation and nomenclature of this biomaterial vary widely amongst authors and are often not well defined. Additionally, they are not uniformly documented in the literature, making results difficult to compare or replicate. In this paper we review the evolution and type of these products available for clinical use. Further we will discuss the scientific rational and technical aspects in preparation of these platelet biomaterials in order to administer them in various fields of medicine.

Minimal residual disease is currently the most powerful prognostic indicator in Precursor B lymphoblastic leukemia. Multiparameter flow cytometry is the most commonly used modality. Seventy three B ALL cases and 15 normal marrows were evaluated for expression patterns of leukemia markers (CD38, CD58, CD73) in all 73 cases and CD66c, CD86 and CD123 in 23 cases. CD73 was aberrantly expressed in 90.41% cases and CD86 in 60.87% B ALL cases. Thus addition of these markers in MRD panels can increase the sensitivity of the assay.