Philadelphia chromosome positive acute myeloid leukemia: a rare hematological disorder: a case report

Philadelphia chromosome positive [Ph-positive] acute myeloid leukemia [AML] is an extremely rare and aggressive disease constituting approximately 1-3% of all de-novo AML cases. This disorder has many features distinct from chronic myeloid leukemia [CML] in blast crisis [CML-BC] and is therefore considered a distinct entity. Patients with Ph-positive AML have lower peripheral basophilia, lower bone marrow cellularity and lower myeloid/erythroid ratio. Presentation is acute with a short history and these patients are less likely to have splenomegaly. Outcome of the disease is poor and median overall survival is 6-9 months. This disease shows resistance to conventional chemotherapy protocols. We have identified two cases of Ph-positive AML amongst all de-novo AML patients diagnosed in our unit from January 2006 to December 2010. Both were treated with two courses of Cytarabine and Daunorubicin followed by Imatinib Mesylate [IM] 600 mg orally daily. Patient no.1 did not respond to two cycles of chemotherapy as well as IM 600 mg daily and died after 5 months. Patient no.2 had a complete hematologic response after two cycles of chemotherapy along with IM and remained in full hematological remission with IM 600 mg daily maintenance for 7 months after diagnosis. After 7 months he had a relapse and died after 2 months of relapse. Combination of AML type of chemotherapy and maintenance with IM provides short term remission while allogeneic stem cell transplant [ASCT] may achieve long term survival in a few patients

Clinico-pathological presentation and therapeutic options for hairy cell leukemia

To look at clinical and hematological presentation as well as treatment outcome of patients with diagnosis of Hairy Cell Leukemia [HCL] in our population. All patients diagnosed with HCL by morphological and immunohistochemical methods presenting to Medical Oncology Ward at Hayatabad Medical Complex, Peshawar since August 2008 were included in the study. Out of 7 patients diagnosed with HCL, 6 were male and 1 was female [M:F ratio 6:1]. Median age at diagnosis was 44 years. Fever, pallor, palpitations and fatigue were the commonest presenting complaints. Spleenomegaly was noted in all patients [100%]. Pancytopenia was noted in all patients at presentation. Five patients were treated with Interferon while 2 were treated with Cladribine. At 18 months of follow-up, one out five patients treated with IFN had relapse while both the patients treated with Cladribine were in complete remission. Our study showed that HCL patients present at a younger age in our region but further studies with larger sample size are required to confirm this. All patients showed a complete response to Interferon and Cladribine with all patients alive at 18 months of follow-up.

Breaking bad news - perceptions of cancer patients and their relatives

To study the perceptions of cancer patients and their relatives regarding disclosure of cancer related information. A cross sectional survey was conducted at Medical Oncology Ward, Hayatabad Medical Complex, Peshawar from September 2009 to January 2011. A total of 114 patients and their relatives were interviewed using a pre-designed questionnaire. Eighty Three [73%] were male while 31 [27%] were female. Median age of cancer patients was 36 [18-70] years. Forty-eight percent [29/60] cancer patients wished for full disclosure of bad news while 39% [21/54] of their relatives wanted full disclosure [p= Not Significant]. Sixty two percent [37/60] cancer patients and 74% [40/54] relatives wanted to be informed in case of recurrence [p= Not Significant]. Over 90% of patients and relatives wanted bad news to be broken by a senior consultant. Fifty eight percent [35/60] cancer patients and 52% [28/54] relatives wished for their relatives to make treatment decisions for them [p= Not Significant]. However, 93% [56/60] of cancer patients and 78% [42/54] of their relatives wanted full information about all side-effects of treatment [p < 0.02]. Fifty eight percent [35/60] cancer patients and 32% [17/54] relatives wanted their close relatives to be present while bad news was being broken [p<0.007]. Almost half of patients wished for full disclosure of information regarding cancer diagnosis. Over 90% patients wanted full information regarding all side-effects of chemotherapy. It is recommended that training regarding how to break bad news be made mandatory part of our medical training both at undergraduate and postgraduate levels

Malignant pleural mesothelioma in northern Pakistan and its response to treatment

Malignant pleural mesothelioma [MPM] is a rare but extremely aggressive malignancy related to exposure to asbestos in over 80% cases. Despite surgery, radiation or chemotherapy, survival is extremely poor. To study the cases of MPM and evaluate their response to chemotherapy Retrospective study at an oncology unit of a major hospital where records were checked over 2 years From January 2001 to December 2002, twelve patients [08 male and 04 female] with histologically proven diffuse MPM were treated with combination of Gemcitabine [1000 mg/m2, IV, day 1 and 8] and Carboplatin [AUC-6, IV, day 1] repeated every 21 days. Age of patients ranged from 50-65 years.All except one patient belonged to the areas adjoining Mardan in NWFP [mesothelioma belt]. Complete responses was seen in none while 4 patients showed partial response and 4 showed stable disease with disappearance of pleural effusion and appreciable improvement in quality life was seen in 8 [66%] patients. Median survival was II months [03 months to 17 months]. Overall, chemotherapy was well tolerated. Gemcitabine and Carboplatin/ Cisplatin offer a chance of palliation with improvement in quality of life and symotoms

Plasma cell leukemia: case report of a rare and aggressive variant of multiple myeloma

Plasma cell leukemia [PCL] is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. We report a patient who presented with history of high grade fever, weakness, palpitations, loss of appetite, bone pains and mental confusion for twenty days. Initial evaluation revealed plasmacytosis with blood plasma cell count of 5184/cumm. His hemoglobin [Hb] was 11.3 gm/dl, platelets were 75000/cumm and total leucocyte count [TLC] was 21600/cumm [24% plasma cells]. Bone marrow examination revealed >60% plasmablasts. Serum LDH was high at 3117 U/L and serum calcium was also elevated at 13.9 mg/dl. A diagnosis of PCL was made and the patient was started on treatment for hypercalcaemia with Melphalan/Prednisolone regime along with supportive care. Patient deteriorated very rapidly despite treatment and died on the eighth day. A detailed report of this case and a review of PCL is presented here

Plasma cell leukemia: case report of a rare and aggressive variant of multiple myeloma

Plasma cell leukemia [PCL] is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. We report a patient who presented with history of high grade fever, weakness, palpitations, loss of appetite, bone pains and mental confusion for twenty days. Initial evaluation revealed plasmacytosis with blood plasma cell count of 5184/cumm. His hemoglobin [Hb] was 11.3 gm/dl, platelets were 75000/cumm and total leucocyte count [TLC] was 21600/cumm [24% plasma cells]. Bone marrow examination revealed >60% plasmablasts. Serum LDH was high at 3117 U/L and serum calcium was also elevated at 13.9 mg/dl. A diagnosis of PCL was made and the patient was started on treatment for hypercalcaemia with Melphalan/Prednisolone regime along with supportive care. Patient deteriorated very rapidly despite treatment and died on the eighth day. A detailed report of this case and a review of PCL is presented here

Remission and survival rates of acute myeloid leukemia patients in Peshawar

Thirty four patients were diagnosed as having acute myeloid leukemia [AML] between July 1994 and June 1996 at Lady Reading Hospital, Peshawar. Aim of the study was to determine the remission rate, leukemia-free survival and overall survival of patients suffering from AML in the center. 20 [58%] patients were male while 14 [42%] patients were female. Median age was 30 years [range 5-59 years]. Twenty one [62%] of our patients were between the age of 17 and 36 years, thus more than half of our patients belonged to the younger age group. M1 and M2 sub-types were the most commonly encountered sub-types of AML in our patients. Minimum follow-up was for 12 months. All 34 evaluable patients received Cytarabine 100mg/m2 for 7 days and Daunorubicin 45 mg/m2 for 3 days [standard 7+3 regime]. Patients achieving a complete remission received four cycles of monthly Cytarabine 100mg/m2 for 5 days and Daunorubicin 45mg/m2 for 2 days [5+2 regime] as maintenance chemotherapy. Daunorubicin was replaced with 6-Thioguanine after 4 months of maintenance therapy. An overall response rate of 62% was achieved [complete remission in 50% patients and partial response in 12% patients]. Leukemia-free survival at 6 months and twelve months was 50% and 30% respectively. Our treatment strategies, results and difficulties involved in the treatment of acute myeloid leukemia patients in developing countries are discussed

Comparison between combinations of tropisetron and dexamethasone with metoclopramide cocktail in the prevention of cisplatin induced emesis

A prospective, randomized, cross-over trial was conducted to compare the anti-emetic effect and tolerability of high dose metoclopramide cocktail with combination of tropisetron and dexamethasone, in patients receiving cisplatin based chemotherapy. Twenty five chemotherapy na‹ve patients receiving cisplatin based chemotherapy were enrolled. 21 [13 male and 8 female] patients were evaluable for analysis. Mean age was 45 years. Mean cisplatin dose was 70 mg/m2. 12 patients [57%] received tropisetron and dexamethasone as first course while 9 patients received metoclopramide cocktail as first course. They were then corssed-over. In the tropisetron group, complete protection in the first 24 hours [acute stage] was 71.5% and 76% respectively. In the metoclopramide group, complete protection against nausea and vomiting in the acute stage was 34% and 28% repectively [p< 0.05 for both]. Tropisetron provided 43% and 52.5% complete protection from nausea and vomiting respectively in the delayed stage while metoclopramide cocktail provide 19% and 33% complete protection only [P=nd. and P< 0.05 resectively]. Side effects in the tropisteron group included mild headache in 3 [15%] and abdominal discomfort in 2 [10%] patients. In the metoclopramide group, 1 patient [5%] had extrapyramidal side-effects and 2 [10%] complained of drowsiness. Tropisetron combined with daxamethasone has superior anti-emetic properties. It is safe, effective and well tolerated