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The article presents a case study of a 34-year-old male who was diagnosed with primary antiphospholipid syndrome (APS) completing the Sapporo criteria, which was diagnosed when he was at age 25. He was also diagnosed with Budd-Chiari syndrome while taking 120 mg of enoxaparin. Moreover, the patient also took prophylactic dose of heparin. The article discusses a treatment for antiphospholipid syndrome through stem cell transplant.

Biphenotypic acute leukemia (BAL) is a rare, difficult to diagnose entity. Its identification is important for risk stratification in acute leukemia (AL). The scoring proposal of the European Group for the Classification of Acute Leukemia (EGIL) is useful for this purpose, but its performance against objective benchmarks is unclear. Using the EGIL system, we identified 23 (3.4%) BAL from among 676 newly diagnosed AL patients. Mixed, small and large blast cells predominated, with FAB M2 and L1 constituting the majority. All patients were positive for myeloid (M) markers and either B cell (B) (17 or 74%) or T cell (T) (8 or 34%) markers with two exceptional patients demonstrating trilineage phenotype. Six (50%) of studied M-B cases were positive for both IGH and TCR. In six (26%) patients myeloid lineage commitment was also demonstrable by electron cytochemistry. Abnormal findings were present in 19 (83%) patients by cytogenetics/FISH/molecular analysis as follows: t(9;22) (17%); MLL gene rearrangement (26%); deletion(6q) (13%); 12p11.2 (9%); numerical abnormalities (13%), and three (13%) new, previously unreported translocations t(X;6)(p22.3;q21); t(2;6)(q37;p21.3); and t(8;14)(p21;q32). In conclusion, the EGIL criteria for BAL appear robust when compared against molecular techniques that, if applied routinely, could aid in detecting BAL and help in risk stratification.Leukemia (2006) 20, 620–626. doi:10.1038/sj.leu.2404128; published online 26 Janaury 2006 [ABSTRACT FROM AUTHOR]

Background Glanzmann thrombasthenia ( GT) is a rare inherited platelet disorder that is characterized by spontaneous or postprocedural bleeding. The diagnosis of GT depends on identifying the dysfunction of the platelets. Aim The aim of this study was to compare a whole blood impedance Multiplate analyzer ( MEA) with the standard method, light transmission aggregometry ( LTA) in diagnosis of GT. Methods Fifteen patients with GT were assessed on MEA and LTA using arachidonic acid ( ASPI: 15 m m), ( TRAP: 1 m m), collagen (100 μg/mL), ADP (0.2 m m), and ristocetin (Risto: 10 mg/mL). Whole blood samples were collected in sodium citrate and hirudin vacuum, blood collection tubes and tested within 4 h. Platelet-rich plasma was used for LTA using platelet agonists (ristocetin 1.5 mg/mL) (arachidonic acid 0.5 mg/mL) ( ADP 2.5 mg/mL) and (collagen 1 mg/mL). Results The platelet count and PFA-100 results were (average and SD) 319 ± 93 × 109 L and 252 ± 34 s, respectively. Flow cytometry analysis showed that all samples are positive for CD42a and CD42b, whereas 9/15 samples were negative for CD61 and CD41. The other six patients had either partial or full expression of CD61/ CD41. Aggregation analysis using both methods showed that all samples had no aggregation response to any of the agonists used apart from six samples which, using only the MEA, showed minimal aggregation in response to collagen (average = 14.3 ± 7 μg, which may suggest ability to detect qualitative abnormality of GPIIb/ IIIa). Conclusion These results suggest that the MEA is sensitive for the detection of Glanzmann thrombasthenia. Furthermore, MEA may also be able to differentiate between the subtypes of Glanzmann thrombasthenia. [ABSTRACT FROM AUTHOR]

The article focuses on study that assess the diagnostic value of antiphospholipid syndrome (aPS) antibodies and compare their utility to that of other aPL antibodies. Topics discussed prospective observational study, diagnostic value of aPS versus other aPL antibodies and independent association between aPS and primary APS.

OBJECTIVE: : Excessive activation of coagulation, which can culminate in overt disseminated intravascular coagulation, is a prominent feature of heat stroke. However, neither the mechanism that initiates the coagulation activation nor its pathogenic role is known. We examined whether the tissue factor/factor VIIa complex initiates the coagulation activation in heat stroke and, if so, whether upstream inhibition of coagulation activation through its neutralization may minimize cellular injury and organ dysfunction. We also examined whether coagulation inhibition influences heat stroke-induced fibrinolytic and inflammatory responses. DESIGN: : Randomized controlled study. SETTING: : Comparative Medicine Department, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. SUBJECTS: : Baboons (Papio Hamadryas). INTERVENTIONS: : Twelve anesthetized baboons assigned randomly to recombinant nematode anticoagulant protein c2, a powerful inhibitor of tissue factor/factor VIIa-dependent coagulation (n = 6), or a control group (n = 6) were heat-stressed in a prewarmed neonatal incubator at 44-47°C until systolic blood pressure fell <90 mm Hg, signaling the onset of severe heat stroke. Recombinant nematode anticoagulant protein c2 was administered as a single intravenous dose of 30 [mu]g/kg body weight at onset of heat stroke. The control group received an equivalent volume of sterile saline intravenously. MEASUREMENTS AND MAIN RESULTS: : Heat stroke was associated with coagulation activation and fibrin formation as evidenced by the increased plasma thrombin-antithrombin complexes, endogenous thrombin potential, and D-dimer levels. Recombinant nematode anticoagulant protein c2 induced significant inhibition of thrombin generation and fibrin formation. Inhibition of coagulation in recombinant nematode anticoagulant protein c2-treated animals did not influence either fibrinolysis (assessed by tissue plasminogen activator, plasmin-[alpha]2-antiplasmin complexes, and plasminogen activator inhibitor) or the release of pro- and anti-inflammatory cytokines. No difference in markers of cell injury and organ dysfunction was observed between recombinant nematode anticoagulant protein c2-treated and control groups. CONCLUSIONS: : Tissue factor/factor VIIa-dependent pathway initiates coagulation activation in induced-heat stroke in the baboon without an effect on fibrinolysis and inflammation. The findings suggest also that coagulation activation is not a prerequisite of cell injury and organ dysfunction. (Crit Care Med 2012; 40:-1236). [ABSTRACT FROM AUTHOR]

There is an increased risk of infection in patients with neutropaenia, especially in those with neutrophil counts of less than 0·5 × 109/L, and neutropaenia-associated infection remains a limiting factor in treating malignancy especially of haematopoietic origin. Transfusing donor neutrophils is a logical approach to these problems, but granulocyte transfusion (GTx), a practice first advocated in the 1960s, is underused and although now enjoying resurgence, remains controversial. The aim of this study was to determine the practical aspects of GTx and clinical responses in patients receiving them. This is an observational retrospective review of GTx in patients undergoing therapy for predominantly haematological malignancies. We reviewed blood bank records and identified patients who received therapeutic granulocytes procured by leukapheresis and linked these recipients with their granulocyte donors. We determined the reasons for GTx and their clinical and relevant haematological responses to the transfusions. We identified 22 patients receiving at least three continuous days of GTx and who had adequate clinical and haematological data. Most donors were relatives and ABO matched with their respective recipients. Mean age of the patients was 28·8 years. Severe aplastic anaemia was the most common diagnosis, occurring in 9 patients (40·9%), followed by acute myeloid leukaemia in 6 (27·3%). Disseminated fungal infection was the most common reason for GTx, occurring in 16 patients (73%), followed by febrile neutropaenia in 7 patients. Fifteen (68·2%) patients showed clinical improvement. This uncontrolled retrospective observational study provides some evidence that procurement and use of GTx is safe for both donors and recipients and is probably an effective supportive therapy for patients with febrile neutropaenia [ABSTRACT FROM AUTHOR]