Summary Peripheral blood lymphocyte sets and serum immunoglobulins were examined in 15 patients with thalassaemia intermedia, aged between 5 and 30 years. They were divided in three clinical groups: Group I, non‐splenectomized, with serum iron (SI) lower than 200 μg/dl; Group II, splenectomized with serum iron lower than 200 μg/dl; and Group III, splenectomized with serum iron levels greater than 200 μg/dl. High absolute lymphocyte counts were observed in the majority of patients, whether splenectomized or not. Following splenectomy, marked increases were observed in absolute number of lymphocytes and percentages of circulating surface immunoglobulin (SIg) bearing B lymphocytes, mouse rosette forming cells (MRFC) and ‘null’ cells. The increases in circulating B lymphocytes following splenectomy were due exclusively to cells bearing SIgM and/or SIgD. No changes were observed in numbers and proportions of circulating SIgA or SIgG bearing cells. Serum IgA levels were high in the majority of patients studied whether splenectomized or not. Following splenectomy increases in serum IgA and serum IgG were observed which seemed to relate to increasing SI levels. No significant changes occurred in serum IgM levels. Absolute numbers of circulating T lymphocytes were unaffected by splenectomy or SI levels. Changes were observed, however, in distribution of T lymphocyte subsets with decreases in number of Tμ cells and concomitant increases in numbers of non‐Tμ, non‐Tγ cells. Tγ cells were the least affected by splenectomy and increasing SI. The results are compatible with the proposition that iron participates in the regulation of lymphoid cell migration and in the modulation of expression of cell surface markers. The findings also illustrate the role of the spleen in the control of lymphocyte migration and immunoglobulin production.
Twenty five patients with beta thalassemia major, with no evidence of infection were evaluated for their polymorphonuclear cell (PMN) metabolic function and serum opsonic activity by chemiluminescence assay. These were divided into Group I of normal adults (n = 21), Group II thalassemia major < 5 years (n = 9) and Group III thalassemia major > 5 years (n = 16). The ability of the chemiluminescence assay (CL) to reflect opsonic and phagocytic dysfunction suggested its potential application in the evaluation of phagocytic function. The peak count of Group I was (1.07 +/- 0.24 x 10(-5)), Group II (1.60 +/- 0.83 x 10(-5)) and Group III was (2.71 +/- 0.98 x 10(-5)) respectively in the presence of autologous sera. The peak count compared between Group I and III was found to be statistically significant (p < 0.05). The peak count of Group I and II when compared showed a trend in the increase activity not statistically significant. The polymorph function of all the groups were compared with autologous serum as well as normal serum. There was no increase in polymorph function of Group III in the presence of thalassemia serum, nor any decrease in the polymorph function of thalassemia patients of Group II and III. This concluded that polymorphs of thalassemia patients are active in the presence of autologous as well as normal serum. The increased activity of thalassemia polymorphs may be due to antigenic stimulation which may be due to multiple transfusion and not due to circulating iron load.
BackgroundDiffuse large B-cell lymphoma (DLBCL) is the commonest subtype of lymphoma, standard CHOP was the treatment of choice, 42% of patients received rituximab, and 29% of patients were lost to follow-up during therapy, were reported in a study that collected retrospective data at 13 public and private hospitals for patients diagnosed with lymphoma between January 2005 and December 2009. The OncoCollect Registry was set up in 2017 to address the challenges in the collection of retrospective data through chart review, recording access to anthracycline and rituximab-based treatment, and to study outcomes and any improvement in the patient follow-up.MethodologyThe OncoCollect Lymphoma group registry was set up at a national level with 9 participating centers. Lymphoma patients registered at these centers between 2011 and 2017 were included. The clinical features, prognostic stratification, associated comorbidities, response to first-line treatment, and 3-year outcomes of adult patients with DLBCL were analyzed.ResultsOf the 5,886 lymphoma patients registered in the OncoCollect registry, 2,581 (44%) had DLBCL. A total of 1,961 were evaluable for frontline therapy. The median age at presentation was 57 years. Gender ratio was 1.6:1. At presentation, 43% were early stage, 70% had low and low intermediate IPI, 53% had extranodal disease, and 30.9% had one or more comorbidities (data available for 1,136 patients). The commonest extra nodal site was gastro-intestinal (23.98%) followed by head and neck (19.24%). The overall response rate was 79.29%. Complete remission was seen in 61.75%, partial response in 17.5%, stable disease in 4.3%, and progression in 7.9%. Patients who received anthracycline-based therapy (86.7%) and rituximab-based therapy (83.7%) had a 3-year event-free survival (EFS) of 69.67% and 68.48%, respectively. With a median follow-up of 33 months, the 3-year overall Survival (OS) and EFS were 75.37% and 66.58%, respectively.ConclusionsDLBCL remains the commonest (44%) lymphoma subtype and is curable with standard anthracycline- and rituximab-based therapies. The availability of rituximab has increased the proportion of patients receiving standard chemoimmunotherapy.
Introduction: Development of DNA-based tests for TPMT/DPD polymorphisms can help clinicians and patients to make important decisions about cancer treatment. Also, due to lack of Indian data, we aimed at the development and validation of these tests in Indian patients. Materials and Methods: Molecular assays were used for identifying TPMT/DPD variations; validated by DNA sequencing. Results: Molecular assays have been used for screening TPMT∗2, ∗3A, ∗3B, ∗3C alleles and IVS14+1(G→A) in DPD gene. A patient, exhibiting neutropenia on 6-MP was observed to be G460A-homozygote, while, two Acute Lymphoblastic Leukemia (ALL) patients with side-effects exhibited wild-type alleles. Two patients showing 6-MP side-effects and responding well to the same drug at later stage also carried wild-type alleles. Discussion: G460A homozygosity in a patient allowed clinicians to stop 6-MP treatment, improving patient's health status. Two ALL patients showing side-effects were wild-type, indicating presence of unidentified rare variations. Two patients with wild-type allele showed side-effects during 6-MP treatment, but responded well to same drug at later stage, suggesting side-effects to be attributable to multiple biological and environmental processes. Absence of IVS14+1(G→A) in DPD gene will not exclude possibility of another mutation. Conclusion: Molecular assays for determining common TPMT/DPD variations, can provide accurate diagnosis and efficient therapies in future clinical studies.
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