Presentation of a patient with palpable purpuric rash

Henoch Shonlein purpura (HSP) is a form of systemic vasculitis characterized by deposition of IgA dominant immune complexes in the small vessels. The triad of palpable purpuric rash on lower extremities, abdominal or renal involvement and arthritis is the typical presentation in this condition. The disease primarily affects children and is less common in adults. We report a case of a young female who presented with palpable purpura on legs, classical symptoms of HSP i.e. arthritis and off and on abdominal pain

Acute transfusion reactions encountered in patients at a tertiary care center

Objective: To determine the frequency and type of Acute Transfusion Reactions (ATRs) occurring in inpatients, reported to the transfusion service at Aga Khan University Hospital, Karachi, Pakistan. Methods: This was a three years and seven months (from January 2005 till July 2008) retrospective review of all the transfusion reactions that were reported to the transfusion service at Aga Khan University Hospital, Karachi, Pakistan. All the reactions were clinically evaluated by the blood bank physician. Transfusion reactions occurring during or within four hours after transfusion were evaluated and classified by standard and recognized definitions defined by American Association of Blood Banks. Results: The acute transfusion reactions (ATRs) reported during the study period were 212. However, out of these 212 ATRs, 182 ATRs were confirmed by blood bank physician, and included febrile non haemolytic reactions [89 (41.9%)], allergic reaction [73 (34.4%)], isolated hypotension [3 (1.4%)], haemolytic reaction [4 (1.8%)] and bacterial contamination [2 (0.9%)]. Eleven (5.1%) ATRs were unclassifiable and were thus labeled as non specific reaction.Conclusion: The frequency of transfusion reactions in our patients was found to be 0.082%. Febrile non haemolytic reaction was the most frequent transfusion reaction followed by allergic reaction. This may be an under reported figure. There is a need for establishing a haemovigilance system for critical analysis of blood transfusion event

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

Follicular Dendritic Cell Sarcoma of Lymph Node - a Rare Entity

Follicular dendritic cells (FDC) are non-lymphoid, non-phagocytic accessory cells in the immune system that are essential for antigen presentation and germinal center reaction regulation1. These cells are CD21+, CD35+, CD1a- and S100 protein + and they show desmosomes ultrastructurally.The most commonly involved sites by FDC tumors are lymph nodes but may arise at a variety of extranodal sites including oral cavity, tonsil, gastrointestinal tract and liver. Most studies represent single case reports or case series. Our patient presented with tumor in the lymph nodes. Histology revealed tumor cells with abundant eosinophilic cytoplasm, hyperchromatic and pleomorphic nuclei, and prominent nucleoli. The tumor cells were found to be positive for CD21 which is a specific marker for follicular dendritic cells

Frequency of hereditary thrombophilia: an AKUH experience.

Abstract Objective: To determine the frequency of various causes of hereditary thrombophilia at a referral laboratory and the age and gender distribution. Methods: This is a descriptive study incorporating a retrospective analysis of requests for thrombophilia screening sent to Clinical laboratory, Aga Khan University Hospital from November 1995 to May 2002.Patients were screened for hereditary causes of thrombophilia including Protein C, Protein S, antithrombin III, Factor V Leiden and homocysteine. Frequency of each disorder; and age and sex distribution was determined. Results: All the patients suspected clinically for thrombophilia were screened. Of the 2825 patients, 70 were diagnosed to have inheritance as a cause of thrombophilia with a frequency of 2.3% for protein C deficiency, 1.4% for protein S deficiency, 1.5% for antithrombin III deficiency, 14.2% for factor V leiden mutation and 2.0% for homocystenemia. Conclusion: All the causes of hereditary thrombophilia can be diagnosed by relatively simple laboratory methods, however because of the low frequency of these disorders the screening of general population is not indicated in the absence of clinical symptoms. More prospective studies are required to define the occurrence of these disorders and other causes of thrombosis (JPMA 54:427;2004)

Frequency and clinical spectrum of rare inherited coagulopathies--a tricenter study

OBJECTIVE: To determine the frequency of rare inherited coagulopathies at three centers of haematology in Karachi and to study the clinical spectrum and laboratory data of these coagulopathies. METHODS: This was a descriptive study conducted from September 2003 to December 2004 on subjects from Aga Khan University Hospital, Husaini Blood Bank and Fatimid Blood Transfusion Centre. All the subjects with bleeding tendency without any acquired causes of bleeding were selected for further investigation, and were asked relevant questions as present in the questionnaire. Screening tests including platelet count, PT, APTT and bleeding time were performed on all patients and subsequently, specific tests including factor assay, clot solubility test, platelet aggregation and vWFAg were performed. RESULTS: In total, 1100 patients were evaluated for bleeding tendency at the three centers and 65 patients were diagnosed to have inherited coagulopathy other than haemophilia A and B. Out of these 65 patients, 33 (50.7%) were males and 32 (49.2%) were females. Rare inherited coagulopathies that were found in our population included deficiency of factor VII {n = 21 (32.3%)}, factor X {n = 17 (26.1%)}, factor XIII {n =14 (21.5%)}, factor V {n = 9 (13.8%)}, fibrinogen {n = 2 (3%)}, prothrombin {n = 1 (1.5%)} and factor XII {n = 1 (1.5%)}. CONCLUSION: Inherited coagulopathies other than haemophilia A and B were noted in the study population

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\u27s 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

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

<b>Objective:</b> 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. <b>Setting:</b> A tertiary care hospital in Karachi, Pakistan. <b>Study Design:</b> This is a retrospective study. <b>Materials and Methods:</b> 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. <b>Results:</b> There were 46 patients diagnosed during the study period and on regular follow-up. Forty five (97.8&#x0025;) of these were in complete remission after 28 days of induction therapy. Thirty patients (65.2&#x0025;) were alive and doing well at the time of study. Of these 30 patients, 26 (86.6&#x0025;) remained relapse free while only four (13.3&#x0025;) had relapsed. The remaining 16 patients (34.7&#x0025;) did not survive including 11 (68.7&#x0025;) who had a relapse. Only significant variables in terms of prognosis were age and ALL phenotype with a <i>P</i> value 0.04 and 0.03 respectively. <b>Conclusion:</b> 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

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

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