Clinico-pathological analysis of lymphoproliferative disorders: a 3 year study

Background: The incidence of lymphoproliferative disorders has increased in many parts of the world. Newer subtypes have been identified by the new WHO classification. Accurate subtyping of lymphomas is crucial for prompt treatment. Objective of the study was to assess the clinicopathological pattern of lymphoproliferative diseases diagnosed in Rajagiri hospital over a period of 3 years.Methods: A retrospective study on all patients who were diagnosed with lymphoma in Rajagiri hospital during January 2016 to December 2018 was conducted and the data were reviewed and analyzed.Results: A total of 151 patients were included in the study. Majority of the subjects (63%) were males. The predominant age group affected was 61-80 years. Mean age group was 58.46 years (SD=19.05 years). Most common presenting symptom was painless lymphadenopathy. B symptoms were seen in 18% of subjects and was found to be more commonly associated with B cell Non-Hodgkin lymphoma. Most common lymph node involved was cervical lymph node, while the most common extra nodal site was bone marrow. Most common lymphoproliferative disorder was Diffuse large B cell Lymphoma. Lymph node involvement was found in 74% of patients, while hepatomegaly and splenomegaly were seen in 11% and 18.5% of patients. 20% of patients had secondary bone marrow involvement. 8 patients developed recurrent lesions involving other organs.Conclusions: Clinicopathologic patterns of lymphoproliferative disorders vary across various regions. A proper understanding of demographical distribution of lymphomas is very essential, as it can provide valuable clues for accurate diagnosis and treatment.

Allogeneic peripheral blood stem cell transplantation in patients with haematological malignancies

To report the initial data on allogeneic peripheral blood stem cell transplantation for haematological malignancies in Pakistan. Design: A single centre descriptive study. Place and Duration of Study: Bismillah Taqee Institute of Health Sciences and Blood Diseases Centre from September 1999 to June 2004. Patients and Patients with haematological malignancies were included who had received allogeneic PBSC transplantation of Filgrastim [rhG-CSF] mobilized peripheral blood stem cells from HLA-identical siblings [except one 5/6 antigen sibling] with Busulphan and Cyclophosphamide standard conditioning therapy in all patients. No patient received antibiotics for gut decontamination. Empirical antibiotics included Ceftriaxone and Amikacin for febrile neutropenia, oral Itraconazole for antifungal prophylaxis while oral acyclovir was used for antiviral prophylaxis. All donors and recipients were CMV IgG positive Cyclosporin A / Methotrexate were given for graft versus host disease [GvHD] prophylaxis. Stem cells were harvested using Haemonetics MCS+ cell separator. All patients received G-CSF starting from day +4 until their neutrophil count rose to normal. There were 21 patients with age range of 8-38 years and male to female ratio of 2:1. Engraftment was achieved in all patients; median time to absolute neutrophil count of > 0.5 x 109 /l was 10 days [range 8 ' 12 days] and platelet count of > 20 x 109 /l was 14 days [12'17 days]. Acute graft versus host disease [aGvHD] was seen in 7 patients; one patient had grade IV skin and hepatic GvHD; another patient had grade III gut GvHD, grade II GvHD was seen in 3 patients while grade I skin aGvHD was seen in 2 patients. Median hospital stay was 34 days. Treatment related mortality was seen in 3 patients [18%]. Chronic GvHD was seen in 5 patients. Four more patients died during the follow-up period. Malaria was seen in 2 while tuberculosis developed in one case. Relapse was seen in 2 patients. The estimated probability of survival at one hundred day, at one year and five years was 82, 47 and 40% respectively. Haematopoietic stem cell transplant programme can be developed in a developed country setting. Post transplant complications are similar to what have been reported in the developed countries. In endemic areas malaria could prove to be fatal if not recognised and treated early

Peripheral blood stem cell transplantation in children with B-thalassemia major

To share the preliminary data on stem cell transplantation in Pakistan. Design: This is a single center retrospective analysis of the outcome of allogeneic peripheral blood stem cell [PBSC] transplantation in b-thalassemia major patients. Place and Duration of Study: Bismillah Taqee Blood Diseases Centre, Karachi; a tertiary care hematology center from September 1999 to September 2002. Patients and Twelve patients with b-thalassemia major received allogeneic PBSC transplantation from HLA identical sibling donors except in one case where mother was the donor. All patients received busulphan and cyclophosphamide conditioning therapy and cyclosporin A / methotrexate for graft versus host disease [GVHD] prophylaxis. Donors were primed with G-CSF for 4 days and stem cells were harvested using Haemonetics MCS + cell separator on 5th day. All patients received G-CSF starting from day + 4 until their neutrophil count rose to normal. Engraftment was achieved in all patients except one who required a second dose of bone marrow graft on day +21. Median time to achieve absolute neutrophil count of > 0.5 x 109 /l was 9.0 days [range 8 ' 31 days] and platelet count of > 20 x 109 /l was 14 days [12 ' 35 days]. Acute GVHD was seen in 3 patients, one patient had grade IV gut GVHD; another patient had grade III gut GVHD while third patient had grade II skin GVHD. Median hospital stay was 29 days. Six patients were well and transfusion independent 3 to 36 months posttransplant. One episode of primary graft failure required a second dose of bone marrow harvest. Another episode of graft rejection received two doses of donor lymphocytes infusion. There were 4 deaths due to grade IV gut GVHD because of uncontrolled systemic Candida infection and one due to hepatic veno-occlusive [VOD] disease. Allogeneic peripheral blood stem cell transplantation can be safely and economically carried out in Pakistan. Although there had been 4 deaths during 36 months follow-up, with increasing understanding and experience the outcome is expected to improve

Application of fibrin glue in maintaining post operative haemostasis in a thrombocytopenic patient: a novel approach case report

Here we report a case of chronic refractory autoimmune thrombocytopenic purpura [ITP] who presented with severe persistent thrombocytopenia, 3 months history of incisional hernia with aggravating symptoms. Considering the potential danger of acute obstruction, with its disastrous consequences, surgery was performed under cover of fibrin glue and hernia was repaired without any excess post operative bleeding