ALBERT

Description: Background Peripheral blood smears from finger stick specimens normally show a variable number of platelet clumps. This is believed to be due to platelet activation during the preparation and exposure of glycoprotein Gp Iib/IIIa receptors. Congenital lack of Gp Iib/IIIa (CD41/61) such as seen in Glanzman’s thrombasthenia is characterized by presence of single unclumped platelets in peripheral blood smears. Gp Iib/IIIa antagonists such as Tirofiban cause Glanzman’s thrombasthenia-like defect and would be expected to inhibit platelet clumping in peripheral blood smears. Cardiologists, for the treatment of acute coronary syndrome, increasingly use Tirofiban or other Gp Iib/IIIa blocker. It is given intravenously and has a short half-life. It is thought to be particularly effective because of inhibition of the final common pathway of platelet aggregation. Methodology/results We retrospectively analyzed from January 2000 to Feb 2005, blood smears from finger stick preparations of 35 patients on Tirofiban and 35 controls, who had normal platelet count, for the presence of platelet clumps. The Tirofiban group was referred to the hematology service by cardiologists, mostly for workup of anemia or presence of hypercoagulable state. The control group was patients in the Hematology- Oncology outpatient, who had diagnosis of non-myeloproliferative disorder. Only patients with normal platelet count were included. Slides were prepared from patients while they were on Tirofiban infusion. This information was based on chart review and computer records. All 70 slides were blinded. Hematology fellows under the supervision of hematology attending physicians counted 200 platelets on each slide at 50x magnification. Platelets were classified as single unclumped platelets, platelets in clumps of 2, 3, 4, or 5 and over. More than 90% of platelets were single unclumped in 28 of 35 patients on Tirofiban, while more than 90% of platelets were single unclumped in only 2 of 35 patients in the control group. Conclusion GpIIb/IIIa antagonists are increasingly used for patients with acute coronary syndrome. The inhibition of platelet clumping in peripheral blood smears of patients on these agents has not been studied. We found a significant decrease of platelet clumping in patients on Tirofiban as compared to controls. This is an example of utilization of knowledge of congenital defect and iatrogenic effect of medication, for possible patient use. Further study is needed to confirm this observation and correlate it with the clinical benefit in patients with acute coronary syndrome. The possibility of GpIIb/IIIa resistance may also need to be looked into. This will be a simple, inexpensive test that may be used to correlate with response or possible need for dose adjustment. This is an extension of a study of 20 patients, which was accepted as an abstract, at the annual American College of Cardiology meeting, in March 2005 in Florida. Tirofiban (35) Control (35) Number of patients with 〉90% single unclumped platelets 28 2 Number of patients with

Description: Background: Lupus Anticoagulants (LA) are antibodies that interfere with phospholipids dependent coagulation pathways. First indication of the presence of LA is usually provided by prolonged aPTT. The sensitivity of various commercial aPTT reagents for LA varies. For some years, trend has been to shift towards aPTT reagents that are more sensitive to LA. Starting from May 2004, our coagulation laboratory changed aPTT reagent from Thrombosil by Hemoliance to aPTT-SP by Hemosil. During transition, for quality control purpose, we continued to perform aPTT with Thrombosil in patients found to have prolonged aPTT with aPTT-SP. The aPTT-SP is considered to be more sensitive for LA than Thrombosil. Since then we found increased frequency of prolonged aPTT being discovered during screening coagulation tests. As per our laboratory protocol, all the samples with prolong aPTT were further evaluated with mixing studies and Dilute Russel Viper Venom Test (DRVTT) for LA. We did a retrospective study to evaluate the clinical significance of prolonged aPTT by aPTT-SP. Method: Retrospective chart review of all the patients with prolonged aPTT with aPTT-SP from May 2004 to April 2005 was done. Information regarding patient’s age, sex, laboratory data, history of any thrombo-embolic phenomenon and other significant medical conditions was collected. Results: In 1 year 50 patients (28 females and 22 males) with age ranging from 19 to 80 years, with prolonged aPTT with aPTT-SP reagent were identified. 29 had prolonged aPTT by both reagents and 21 had prologed aPTT by aPTT-SP only. 13 of 21 patients with prolonged aPTT with aPTT-SP (more sensitive reagent) and normal aPTT with Thrombosil (less sensitive reagent), had positive LA. Only three had vascular thrombosis. Most institution including ours, routinely tests for LA during thrombophilia work up regardless of aPTT result. Thus LA would have been diagnosed in these three patients despite normal aPTT by Thrombosil. At the same time diagnosing LA in the absence of thrombosis does not lead to change in clinical management. Results are shown in table. Conclusion: Use of aPTT reagent more sensitive to LA, does not provide any clinical advantage. In fact it led to extra work-up and delay in invasive procedures. It also had the potential to lead to extra intervention, like administration of FFP in emergency situations, before LA as a cause of prolonged aPTT could be determined. results S. No VARIABLES PATIENTS=n * 2 had venous thrombosis, 1 had coronary artery disease.**3 had venous thrombosis, 2 had CVA, 2 had coronary artery disease.*** The reasons for prolonged aPTT by both reagents were varied like coumadin intake, LMWH, 1 patient had factor 11 deficiency etc 1 Increased aPTT by aPTT SP/Normal aPTT by Thrombosil/LA Negative 8 2 Increased PTT by aPTT SP/Normal aPTT by Thrombosil/LA Positive/No Thrombosis 10 3 Increased aPTT by aPTT SP/Normal aPTT by Thrombosil/LA Positive /H/o Thrombosis 3* 4 Increased aPTT by both Reagents/LA Positive/H/o Thrombosis 7** 5 Increased aPTT by Both reagents/LA Positive/No Thrombosis 12 6 Increased aPTT by both Reagents/LA negative 10***

Description: Background - Priapism is a urologic emergency caused by hematological problems in about 35% of cases. Its occurrence in sickle cell anemia, leukemia /other malignancies and primary thrombocythemias is well established. A very few case reports of priapism occurring in Thalassemia Intermedia especially after splenectomy have been documented in literature. Veno-occlusive priapism (low flow priapism) beyond 4hours is a compartment syndrome requiring emergent medical intervention. The most important late complication of priapism is fibrosis and impotence and their incidence is related to the duration and aggressiveness of treatment of priapism. We report a case of priapism in a patient of Thalassemia minor. Case report- A 42 year Asian male was referred from urology service for a hematologic work-up for recurrent episodes of priapism. He was admitted with a 24 hours-long episode of priapism. The history of priapism dates back to 1998, a few months after marriage, when he was admitted for the first episode of priapism and had a decompressive procedure. Since then, for 6 years, he had intermittent, 15 minutes to 1 hour episodes of penile erection. These episodes usually occur after midnight and were unrelated to sexual activity. He did not have any other medical problems and was not on any medications. He was not on any pro-erectile medications. He denied any history of masturbation. He denied any history of alcohol or illicit drug use. He was unaware of any family history of anemia. On physical examination, he did not have pallor, icterus, lymphadenopathy, skin ulcers or hepatosplenomegaly. The penis was circumcised without any trauma or necrosis. His hemoglobin was 10.5 g/dl, hematocrit 34%, MCV 65fl, RDW14.5 %, reticulocyte count 1.3%, platelets 274k/μl, and WBC 6.9k/μl. Peripheral smear showed poikilocytosis, hypochromia and microcytosis without any sickle cells or nucleated RBCs. Hemoglobin electrophoresis showed Hb A of 94%, Hb A2 of 5.6% and Hb F of 0.4%. His clinical picture, blood count, peripheral smear and electrophoresis were consistent with Thalassemia minor. He was managed by removal of inspissated blood and injection of phenylephrine into the corpora cavernosa. This was repeated a few times and the priapism was relieved and patient discharged. Conclusion- This is the first reported case of priapism in a patient with Thalassemia minor.

Description: Background : The Lupus anticoagulant is known to prolong coagulation tests, however the source of antibody causing this prolongation is not clear in the majority of patients. We are reporting a patient with prolongation of prothrombin time (PT) and activated partial thromboplastin time (aPTT) secondary to a lupus anticoagulant like activity of an IgM paraprotein. Case report : An 82 years old male patient was referred in January 1996 to our clinic for abnormal PT and aPTT. He was asymptomatic. There was no lymphadenopathy or organomegaly. The hemoglobin was 10.4 (g/dl) with normal white cell and platelet count. Renal and liver function tests were normal. Coagulation studies revealed aPTT 145.4 seconds, the PT 38.8 seconds, thrombin time 24.9 seconds, fibrinogen level of 336 mg/dl and D Dimer 3.1 and 1:500 dilution 〉2.9 (normal 106 seconds to 48 seconds upon the addition of platelet phospholipids (a difference of 〉5 sec is taken as positive for LA). Expanded antiphospholipid antibodies test were done. Anti-cardiolipin IgM (〉100MPL), anti-phosphotidyl serine IgM (7.3SD) were detected. A serum protein electrophoresis revealed monoclonal gamma spike of approximately 〉 4gm/dl and quantitative immunoglobulin by radial immuno-diffusion (RID) showed IgG- 1691 mg/dl, IgA - 185 mg/dl, IgM 〉192 mg/dl. The IgM 〉192 mg/dl is limited by the linearity of the test. IFE (Immunoelectrophoresis) demonstrated IgM-λ spike. Serum viscosity was 3.4. We suspected that the IgM paraprotein might be the inhibitor. IgM was purified by Euglobulin flocculation test. The PT and aPTT of normal pooled plasma was prolonged on its addition. The conclusion was that antiphospholipid activity was in IgM. Bone marrow biopsy in January 1996 showed