Waldenstrom\u27s macroglobulinemia terminating in acute myeloid leukemia

Waldenstrom\u27s macroglobulinemia (WM) is a rare condition, accounting for approximately 2% of haematologic malignancies. The most common causes of death in these patients are progression of the malignant lymphoproliferative process, infection and cardiac failure. Acute leukemia is a rare event in the clinical course of WM. A number of case reports have documented the development of terminal acute leukemia in patients with WM following prolonged chemotherapy

Endocrine complications in patients of beta thalassemia major in a tertiary care hospital in Pakistan

OBJECTIVE: To determine the major endocrine complications present in patients of Thalassemia major presenting to a tertiary care center in Karachi, Pakistan. METHODS: Records of all thallasemia major patients at a Haematology Department in a tertiary care hospital of Karachi were retrospectively reviewed from May to August 2009 with specific focus on endocrine data and investigations. The patients\u27 data was recorded in a questionnaire and analyzed using SPSS v.17, frequencies and percentages along with corresponding 95% confidence interval were computed. RESULTS: Our data revealed that a significantly small percentage of those under care received regular endocrine follow-up. Male hypo-gonadal abnormalities had the highest probability; 100% of the tested patients had decreased levels of testosterone, while 95.2% had raised serum creatinine levels. Parathyroid dysfunction was noted in 40% of the patients. Of those screened 29.4% had blood glucose levels in the diabetic range and 11.8% of the tested patients had reduced levels of FT4. CONCLUSION: A high frequency at endocrine dysfunctions seen in thalasaemia patients included: male hypogonadism, parathyroid dysfunction, deranged blood glucose and FT4 levies

Old disease, new targets. Part-II, haematological malignancies

In last two decades newer therapies in cancer treatment have emerged and have opened new horizons. New term of targeted therapy has emerged and for certain malignancies the paradigm has really changed after the introduction of these agents. We have learnt and have seen the outcome of some diseases after the addition of these monoclonal antibodies (MoABs) and tyrosine kinase inhibitors (TKIs). Rituximab a MoAB against CD-20 has really paved its role in the treatment of B-cell lymphomas and become the sort of standard therapy. The TKIs are newer agents available in a pill form and have inhibited many pathways at cellular level which are necessary for cancer development. Imatinib has really changed the prognosis and outcome of chronic myeloid leukemia (CML) remarkably. For those patients who develope intolerance to imatinib or their disease became resistant to the imatinib the newer agents like dasatinib and nilotinib are second line options. The major edge of these newer agents is more potency with low side-effect profile. The major concern remains the cost

Cerebral venous sinus thrombosis in a patient with undiagnosed factor VII deficiency

Factor VII (FVII) deficiency is one of the rare inherited bleeding disorders. Thrombosis has been occasionally described in inherited FVII deficiency. Here, we report a young female with undiagnosed FVII deficiency who presented with cerebral venous sinus thrombosis (CVST). Oral contraceptive pill was found to be prothrombotic risk factor. The CVSToccurred in spite of the congenital FVII deficiency indicating that no definitive antithrombotic protection is assured by this defect. Low molecular weight heparin and anti-Xa assay were found to be safe choice of anticoagulation and monitoring, respectively, in this patien

Autoimmune hemolytic anemia in visceral leishmaniasis

A case of an 8-month old male child, who presented with lgG mediated Coomb’s positive hemolytic anemia and visceral leishmaniasis is presented. The hemolytic anemia resolved following the treatment of leishmaniasis. Although various other mechanisms of anemia have been described, so far the association between IgG-mediated hemolytic anemia and visceral leishmaniasis has not been reported

Oral iron chelation therapy with deferiprone in patients with Thalassemia Major

OBJECTIVE: To determine the efficacy and adverse effects of deferiprone in patients with Thalassemia Major. METHODS: A prospective case series study was conducted at the Fatimid Foundation Blood Bank and Haematological Diseases center Lahore. A total of 87 patients entered into the study between September 2005 and November 2006. Deferiprone was given at subsidized rates at a dose of 75/mg/day for seven days. Physical examination and initial Laboratory investigations were done in all patients at the start of the study. Physical and laboratory data were filled on a questionnaire and analyzed using SPSS version 10.0. RESULTS: Eighty seven patients with mean age of 10.0 +/- 4.33 years (range 4-27 years) were included in the study. Mean follow up was 8 +/- 3.94 months (range 2-12 months). The mean Ferritin at the start of study was 4656 +/- 2052.5 ug/L (range 1200-14630 ug/L) and at the end of study period was 4139 +/- 1710.4 ug/L (range 749-8961) (p \u3c 0.001). Adverse events were joint pains in 10% patients, gastrointestinal symptoms in 11% and no adverse events in 79% patients. There was no evidence of agranulocytosis in any patient. CONCLUSION: Deferiprone was well tolerated, had few adverse effects and was effective in lowering the patient\u27s serum ferritin level

Metastatic signet ring gastric adenocarcinoma presenting with microangiopathic hemolytic anemia.

Metastatic adenocarcinoma presenting as microangiopathic hemolytic anemia (MAHA) and leukoerythroblastic blood picture is rare. We report three patients who presented with MAHA as the initial symptom of metastatic signet ring cell gastric adenocarcinoma. One patient had past history of gastric ulcer. In all these patients the initial diagnosis was based on peripheral blood smear followed by bone marrow biopsy; upper GI endoscopy showed presence of gastric ulcers with focally scattered neo-plastic signet ring cells on histopathology. All patients died within a week of diagnosis

Autologous hematopoietic stem cell transplantation-10 years of data from a developing country

Intensive chemotherapy followed by autologous stem cell transplantation is the treatment of choice for patients with hematological malignancies. The objective of the present study was to evaluate the outcomes of patients with mainly lymphoma and multiple myeloma after autologous stem cell transplant. The pretransplant workup consisted of the complete blood count, an evaluation of the liver, kidney, lung, and infectious profile, chest radiographs, and a dental review. For lymphoma, all patients who achieved at least a 25% reduction in the disease after salvage therapy were included in the study. Mobilization was done with cyclophosphamide, followed by granulocyte colony-stimulating factor, 300 g twice daily. The conditioning regimens included BEAM (carmustine, etoposide, cytarabine, melphalan) and high-dose melphalan. A total of 206 transplants were performed from April 2004 to December 2014. Of these, 137 were allogeneic transplants and 69 were autologous. Of the patients receiving an autologous transplant, 49 were male and 20 were female. Of the 69 patients, 26 underwent transplantation for Hodgkin\u27s lymphoma, 23 for non-Hodgkin\u27s lymphoma, and 15 for multiple myeloma and 4 and 1 for Ewing\u27s sarcoma and neuroblastoma, respectively. The median age ± SD was 34 ± 13.1 years (range, 4-64). A mean of 4.7 * 10⁸ ± 1.7 mononuclear cells per kilogram were infused. The median time to white blood cell recovery was 18.2 ± 5.34 days. Transplant-related mortality occurred in 10 patients. After a median follow-up period of 104 months, the overall survival rate was 86%. High-dose chemotherapy, followed by autologous stem cell transplant, is an effective treatment option for patients with hematological malignancies, allowing further consolidation of response