Reaction to anti-D immunoglobulin - can we manage it?

Approximately one in six women are blood group RhD negative and are offered anti-D immunoglobulin prophylaxis to prevent sensitization and decrease the risk of haemolytic disease of the newborn in subsequent pregnancies. It has been thought that anti-D is harmless, but there is a risk of anaphylaxis. We describe a case of a woman with a possible immunological reaction to anti-D in her first pregnancy.

Severe prekallikrein deficiency associated with homozygosity for an Arg94Stop nonsense mutation.

An elderly patient with no abnormal bleeding presented with prolongation of the activated partial thromboplastin time (aPTT). Preincubation of plasma with aPTT reagent caused shortening of the abnormal clotting time. Plasma prekallikrein (PK) activity and antigen were <5 u/dL.

Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency.

Background: The pentad of thrombocytopenia, hemolytic anemia, mild renal dysfunction, neurologic signs, and fever, classically characterizes the syndrome of thrombotic thrombocytopenic purpura (TTP). TTP usually occurs in adults as an acquired form but a congenital form in children has also been described. In the latter case, the initial presentation is often with neonatal jaundice and thrombocytopenia.

Thrombotic thrombocytopenic purpura following stem cell transplantation.

Thrombotic thrombocytopenic purpura (TTP) occurring after stem cell transplantation is poorly understood. The literature is scant and heterogeneous; little is known about the condition's pathogenesis except that it appears to differ from that of classical or de novo TTP. There are no widely agreed diagnostic criteria hence, it is difficult to compare the major findings of the relatively small, single centre series that have been reported.

Successful treatment of congenital thrombotic thrombocytopenic purpura using the intermediate purity factor VIII concentrate BPL 8Y.

There is increasing evidence that congenital thrombotic thrombocytopenic purpura (TTP) is caused by an absolute deficiency of von Willebrand factor-cleaving protease. The recent identification of this protease and the development of assays for its detection have enabled its quantification in a number of plasma products, including some commercial intermediate-purity plasma-derived factor VIII preparations. We report the successful, weekly prophylactic use of a commercial intermediate-purity plasma-derived factor VIII concentrate in the treatment of a 14-year-old girl with severe congenital TTP who had previously required transfusions of fresh-frozen plasma every 2 weeks from the age of 4 months.

Microbial overgrowth in water perfusion equipment for esophageal/rectal motility.

Background: There are few data on microbial levels in water used during the assessment of GI motility. Patients undergoing such procedures may be ingesting water with unacceptably high levels of bacteria.

Methods: Samples of water from the reservoir and tubing from water perfusion motility equipment were taken and quantitatively assessed to determine the concentration of viable aerobic and facultative microorganisms.

Von Willebrand factor--cleaving protease activity in congenital thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, fluctuating neurological impairment, renal dysfunction and fever. Both acquired and congenital forms are recognized. Recurrent episodes, which may be predictable (occurring every 21-28 d), are seen in congenital disease and may be treated by infusion with fresh-frozen plasma (FFP).

Identification of the t(15;17) in AML FAB types other than M3: evaluation of the role of molecular screening for the PML/RARalpha rearrangement in newly diagnosed AML. The Medical Research Council (MRC) Adult Leukaemia Working Party.

Acute promyelocytic leukaemia (APL) is characterized by the t(15;17) leading to the formation of PML-RARalpha and RARalpha-PML fusion genes; this rearrangement has been considered both diagnostic for, and restricted to, this subtype of acute myeloid leukaemia (AML FAB M3). We describe two cases of AML with the t(15;17) associated with a PML/RARalpha rearrangement which lacked typical APL morphology, classified as FAB M1 and M2 respectively. In both cases morphological review revealed small populations of cells which exhibited some features associated with APL.