Malignant lymphomas in Pakistan according to the WHO classification of lymphoid neoplasms.

OBJECTIVE: To determine the spectrum of malignant lymphomas in our set up, according to the WHO classification. METHODS: All the cases diagnosed as malignant lymphoma, during the year 2005, were retrieved from the institution based tumour registry record and classified according to WHO criteria depending on the immunohistochemical results of a panel of lymphoma markers. RESULTS: The male to female ratio was 2.5:1 for almost all types of malignant lymphomas. The age range was 3 to 80 years. The frequency of Hodgkin's lymphoma, Burkitt's lymphoma and lymphoblastic lymphoma were higher amongst the children, whereas follicular lymphomas, mantle cell lymphoma and CLL/SLL were more frequently reported in 5th, 6th and 7th decades. Of the total cases 62% were nodal and 38% extranodal (majority in the GI tract). Non Hodgkin's lymphoma was more (73%) frequent than Hodgkin's disease. Mixed cellularity and nodular sclerosis were the main histological variants of Hodgkin's disease. CONCLUSIONS: Immunohistochemistry is not very frequently used in our set up and also at very few other centres. Therefore, its application should be encouraged to raise the quality of data on lymphoid neoplasms and contribute to their control.

Clinicopathological features of lupus anticoagulant; experience at AFIP during last seven years.

BACKGROUND: The lupus anticoagulant (LA) belongs to family of immunoglobulins, most commonly an IgG isotype. These antibodies have been identified most frequently in association with thromboembolic events, recurrent fetal loss and thrombocytopenia. The aim of the present study was to evaluate the presenting clinical and pathological features in patients LA positive presenting at AFIP Rawalpindi over the period of Jan 1993 to Nov 2000. MATERIAL/METHODS: Retrospective analysis of patients presenting with positive LA was carried out. RESULTS: 1583 suspected cases were screened for LA including 1370 females and 213 males. 1024 cases presented with history of recurrent abortions, 292 with thrombosis, 152 with thrombotic strokes before the age of 45 years, 52 with thrombocytopenia and 63 with miscellaneous disorders. Out of 1024 patients tested for recurrent abortions, 130 (13%) females were positive for lupus anticoagulant. Ten (6.5%) of 152 patients presenting with strokes were found positive. Out of 292 cases presenting with thrombosis 17 (5.9%) were found positive. CONCLUSIONS: The lupus anticoagulant prevalence in Pakistani patients with recurrent fetal loss, stroke and thrombosis is statistically significant. Clinicians should be made aware of association of LA with various diseases. The successful management of these patients depends upon close liaison with obstetricians, physicians and haematologists.

Efficacy and adverse effects of oral iron chelator deferiprone (l1, 1,2- dimethyl-3-hydroxypyrid-4-one) in patients with beta thalassaemia major in Pakistan.

BACKGROUND: Deferiprone (DFP,L1) is a bidentate oral iron chelator which binds to iron in a 3:1 ratio. It has the potential advantage of reduced cost and increased compliance. We conducted a study in order to determine the efficacy and adverse effects of DFP in Pakistani thalassaemic patients. METHODS: A group of 26 thalassaemic patients entered the study during the period Jan 1999 to Aug 2002. DFP supplied by Lipomed, Switzerland was given at a daily dose of 75 mg/kg/day (range 50-75 mg/kg/day). After giving informed written consent all the patients were subjected to clinical examination and investigations for monitoring the response. Blood complete picture, liver function tests, blood urea & creatinine, antinuclear factor antibodies (ANF) were tested in all cases before starting DFP treatment. RESULTS: The patients ages ranged from 11 to 27 years, 16 were male and 10 were female. Initial serum ferritin level ranged from 3100-8800 microg/l, mean serum ferritin level was 7129 +/- 1467 microg/l (95% CI 6536 - 7721 microg/l). ECG and Echocardiography was performed in all cases and in 11 cases Cardiac Multigated acquisition (MUGA) Scan was also performed and six patients with impaired left ventricular function were identified. Four patients were lost to follow up and one patient died due to cardiomyopathy. Among the remaining 21 patients serum ferritin levels dropped to 1900 microg/l to 5600 microg/l with mean level of 4288 microg/l (95%CI 3874 - 4702 microg/l), SD 911 microg/l. Significance of difference was (p < 0.001) by Paired samples 't' test. Six patients had gastrointestinal symptoms along with two having arthropathy. ANF positivity was not detected in any patient while on DFP treatment. Similarly, agranulocytosis was not detected in any patient. CONCLUSION: Mean serum ferritin level estimated at the start of trial was 7129 microg/l. This shows that Pakistani thalassaemic patients are quite iron overloaded due to socioeconomic reasons that are peculiar to our setup. In this study DFP was well tolerated and caused fewer side effects. It had much better patient compliance and was effective in lowering serum ferritin level in previously most poorly chelated patients.

Evaluation of LeVeen-shunt patency using Tc-99m labelled macroaggregated albumin.

A LeVeen peritoneo-venous shunt is placed for intractable ascites. Determination of obstruction site in the shunt tube is a difficult problem. We describe a simple nuclear medicine method using 111MBq (3mCi) of Technetium-99m labeled macro-aggregated albumin injected intraperitoneally.

Frequency and clinical importance of anti-Tg auto-antibodies (ATG).

OBJECTIVE: To note the frequency of anti thyroglobulin autoantibodies (ATG) and its clinical importance in 25 follow-up cases of differentiated thyroid cancer (DTC). DESIGN: A case control study. PLACE AND DURATION OF STUDY: The total duration of study was one year (September 2000 to August 2001). Majority of the patients included were the routine follow-up cases at IRNUM, Peshawar. However, few of the cases were also included from NORI, Islamabad and AFIP, Rawalpindi. SUBJECTS AND METHODS: All the patients who had undergone sub-total or total thyroidectomy followed by I-131 ablation therapy were selected for this study. Thyroglobulin (Tg) and ATG were measured using immunometric assay technique with reference range of non-detectable to 40 IU/L. Patients with serum Tg level 10 ng/mL were included in group-1 (n=15) and all the remaining (n=10) in group-2. RESULTS: Overall, 11 patients showed ATG titer above the pre-defined threshold level. In group-1 patients, 8 had positive anti-Tg antibodies in their sera while in group-2, it was positive in only 3 cases. Risk of relapsing metastatic/recurrent disease in association with ATG was calculated which showed that patients with positive ATG have almost seven-fold increased risk of having recurrent/metastatic disease than those who do not. CONCLUSION: Samples for s-Tg measurements must also be evaluated for ATG status because more than one-third of these patients have positive ATG titer in their sera. Although in the presence of positive ATG, the risk of concurrent metastatic/recurrent thyroid disease is increased but still more studies are required to support its significance.