Naturally occurring anti M complicating ABO grouping.

Anti M is considered a naturally occurring antibody that is usually active at temperatures below 37˚C and is thus of on clinical significance. This antibody, if present in an individual, can lead to a discrepancy between forward and reverse ABO grouping and thus creates diagnostic difficulties for blood bank staff. We report a case of a 58-year-old lady who had an unexpected reaction in reverse grouping due to anti M that posed a problem for us in the significance of such discrepancy in blood grouping.

Retrospective review of pediatric patients with acute lymphoblastic leukemia: A single center experience.

Objective: We reviewed the clinical details and treatment outcome of children with newly diagnosed acute lymphoblastic leukemia (ALL) to determine the significance of already established prognostic factors in our patients. Setting: A tertiary care hospital in Karachi, Pakistan. Study Design: This is a retrospective study. Materials and Methods: Children diagnosed with ALL were evaluated over a period of 17 years (January 1, 1989 to December 31, 2006). Data was collected by reviewing the medical records of the patients and the prognostic factors analyzed by us include age, gender, white blood cell count, central nervous system and mediastinal involvement at presentation, morphology and immunophenotype of the blast cells, and response to induction therapy. Results: There were 46 patients diagnosed during the study period and on regular follow-up. Forty five (97.8%) of these were in complete remission after 28 days of induction therapy. Thirty patients (65.2%) were alive and doing well at the time of study. Of these 30 patients, 26 (86.6%) remained relapse free while only four (13.3%) had relapsed. The remaining 16 patients (34.7%) did not survive including 11 (68.7%) who had a relapse. Only significant variables in terms of prognosis were age and ALL phenotype with a P value 0.04 and 0.03 respectively. Conclusion: We found that ALL is a frequent childhood hematological malignancy in our setting and is more prevalent in males and children less than ten years of age. Age and leukemia phenotype emerged as the important prognostic factors in pediatric ALL in our patients.

Clinical audit of inherited bleeding disorders in a developing country.

Objective: We did a clinical audit to determine the status of coagulation disorders in a hemophilia care center in Pakistan. Setting: Fatimid foundation blood bank and hematological diseases center, Lahore. Study Design: This is a retrospective descriptive study. Materials and Methods: All patients registered at Lahore center were included. Data was collected using a questionnaire including age, gender, diagnosis, hepatitis and human immune deficiency virus (HIV) status, number of episodes of bleeding, most common site of bleeding, severity of disorder and number of transfusions required to treat the episode. Results: During the study period, a total of 923 registered patients were reviewed at Lahore center and of these, 408 patients (44.2%) were on regular follow-up. Inherited bleeding disorders identified in these patients included hemophilia A, hemophilia B, vWD, factor VII deficiency, factor V deficiency, factor X deficiency, dysfibrinogenemia, afibrinogenemia, factor XIII deficiency; and platelet function defects. Median age was 17 years with a range of three to 57 years. Median age at diagnosis was one year. There were 329 (80.6%) males and 79 (19.3%) females. The products used in these patients included factor VIII concentrate, fresh frozen plasma, cryoprecipitate, cryosupernatant and platelets. Testing for transmission of viral infections was also done in these patients and one patient (0.2%) was found hepatitis B positive, six patients (1.4%) were hepatitis C positive and two patients (0.49%) were HIV positive. Conclusion: Hemophilia A, hemophilia B and vWD are the commonly encountered inherited bleeding disorders in our patients followed by other recessively transmitted disorders with a median age of 17 years and male to female ratio of 4: 1. Most of the patients utilized services available at Fatimid foundation with good clinical results. In Pakistan, non-governmental organizations (NGOs) are trying their best for providing optimal treatment to patients with inherited bleeding disorders. There is a need for government participation to improve the availability of current hemophilia care services.

Pattern of chromosomal abnormalities in adult acute lymphoblastic leukemia.

OBJECTIVES: To study the pattern of chromosomal abnormalities in adult patients with acute lymphoblastic leukemia. STUDY DESIGN: A retrospective study. Place and duration of study: January 1998 to June 2005 at the Cytogenetics department, Aga Khan University Hospital, Karachi. PATIENTS AND METHODS: A retrospective analysis of cytogenetic studies was carried out in patients who were diagnosed as ALL and were more than 15 years of age. Cytogenetic analysis was performed using a trypsin-Giemsa banding technique. Karyotypes were interpreted using International System for Cytogenetics Nomenclature (1995) criteria. RESULTS: The requests were received for cytogenetic analysis of bone marrow specimens in 69 patients who were diagnosed as ALL. Cytogenetic results were available in 62 patients; out of which 51 were males and 11 were females. 44 patients (70%) were found to have a normal karyotype. In 18 patients (29%), abnormal karyotype was found. CONCLUSION: Cytogenetic studies should be part of the initial work up of every patient with ALL. Larger scale studies will help refine our understanding of the less common chromosomal patterns and conduct multivariate analysis to define the relative prognostic value of karyotypic results.

Granulocytic sarcoma in the absence of acute myeloid leukemia: a case report.

Granulocytic sarcoma is an extramedullary tumor composed of immature granulocytic precursor cells. The most common sites of presentation are bone, periosteum, soft tissue, lymph node, skin, and infrequently small intestine. The tumor may develop during the course of acute myeloid leukemia, chronic myeloid leukemia or other myelodysplastic disorders. It can occur without blood or bone marrow manifestations of leukemia and in this case, the diagnosis is difficult. Our patient was initially diagnosed as a case of T-cell non Hodgkin's lymphoma and received one cycle of CHOP with only transient improvement in his symptoms. Subsequently, his biopsy slides were reviewed at our centre and were reported as granulocytic sarcoma.