Fresh frozen plasma as a source of cholesterol for newborn with Smith-Lemli-Opitz syndrome associated with defective cholesterol synthesis.

The Smith-Lemli-Opitz Syndrome (SLOS) is an autosomal recessive condition that is characterized by a mutation in the DHCR7 encoding the 7-dehydrocholesterol-Δ7 reductase, the enzyme that catalyzes the last step in cholesterol biosynthesis. The syndrome occurs in 1:20,000 newborns with an estimated gene carrier frequency in US Caucasian population of 1 to 2%. The severe form of SLOS in newborns leads to multiple malformations and mental retardation.

Therapeutic plasma exchange and intravenous immunoglobulin as primary therapy for D alloimmunization in pregnancy precludes the need for intrauterine transfusion.

Background: Maternal D alloimmunization detected in early gestation requires aggressive intervention to prevent severe fetal anemia. An intrauterine transfusion (IUT) is indicated to prevent fetal death once severe fetal anemia has been detected, but is not without risk. Protocols combining therapeutic plasma exchange (TPE) and intravenous immunoglobulin (IVIG) have been described, but they usually bridge to IUT.

Treatment of Late Class II Antibody-Mediated Rejection Status Postkidney Transplantation: Two Case Reports.

We are describing the successful treatment of two cases of late Class II antibody mediated rejection status postkidney transplantation. The first patient was treated with a combination of plasmapheresis, intravenous immunoglobulin (IVIG), and stenting of the transplanted renal artery. The second was treated with IVIG and pulse steroids.

Thrombotic thrombocytopenic purpura with unusual 33 recurrences: a case report.

The hereditary or acquired deficiency of ADAMTS-13 activity leads to an excess of high molecular weight von Willebrand factor multimers in plasma, leading to platelet aggregation and diffuse intravascular thrombus formation, resulting in thrombotic thrombocytopenic purpura (TTP). We report a 36 year old male with a long history of TTP associated with 33 relapses. As a result of early transfusions, the patient acquired Hepatitis C.

Should we be screening for anti-Js(a)?

We analyzed our historic patient database at North Shore University Hospital and determined both the overall frequency of anti-Js(a) and the frequency at which it was detected in combination with other alloantibodies to red blood cell (RBC) antigens. Screening cells used currently are negative for Js(a). Our data suggest that anti-Js(a) would not be detected in 30 to 40 percent of patients in which it is the sole antibody present.

When anti-G and anti-C antibodies masquerade as anti-D antibody.

We describe a case of a pregnant woman with anti-C/anti-G antibodies masquerading as anti-D antibodies. Further, confirmation of anti-D antibody is recommended with adsorption-elution studies to confirm the true antibody status. This will avoid the consequence of withholding Rh immunoglobulin prophylaxis in cases when anti-D antibodies are not present.

Unusual warm autoimmune hemolytic anemia in non-alcoholic steatohepatitis.

Warm autoimmune hemolytic anemia (WAIHA), a rare disease (0.2-1 per 100,000 population), ranges from an indolent form with mild hemolysis to a life-threatening condition that necessitates transfusion of incompatible red cells. WAIHA can be either idiopathic or secondary to medications or to a lymphoproliferative disorder.

Tacrolimus (FK506) associated thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in lung transplant salvage with a plasmapheresis and cyclosporin.

Thrombotic microangiopathy (TMA), a microvascular hemolytic disorder is a rare, but well described complication in organ transplant patients receiving immunosuppressant drugs. We report a 56-year-old female with a history of left lung transplant that presented to the hospital with microangiopathic hemolytic anemia and thrombocytopenia while receiving tacrolimus (FK 506) for 36 months. The patient was diagnosed with tacrolimus-induced TTP/HUS and started on daily plasmapheresis, and replacement of FK506 with cyclosporine.

Overt immediate hemolytic transfusion reaction attributable to anti-Wr(a).

Wr(a) is a low-prevalence antigen. Anti-Wr(a) is a relatively common antibody present in approximately 1 in 100 healthy blood donors. Anti-Wr(a) is reported to cause different degrees of hemolysis in transfusion and in HDN, ranging from benign to severe.

Severe hemolytic disease of the newborn in a group B African-American infant delivered by a group O mother.

Maternal-fetal ABO incompatibility is a common hematological problem affecting the newborn. In general, hemolysis is minimal and the clinical course is relatively benign, rarely causing the escalating levels of hyperbilirubinemia and significant anemia commonly associated with Rh hemolytic disease of the newborn (HDN). The incidence of HDN ranges from one in 150 births to 1:3000 births, depending on the degree of anemia and level of serum bilirubin.

Red blood cell exchange transfusion as an adjunct treatment for severe pediatric falciparum malaria, using automated or manual procedures.

Pediatric falciparum malaria is associated with high morbidity and mortality rates. Cerebral malaria and renal failure are common among children with a high percentage of malaria-infected red blood cells. We report 3 cases of imported pediatric falciparum malaria with central nervous system involvement and/or renal failure that were treated initially with intravenous antimalarial therapy, with no clinical improvement.

Surface decoration of red blood cells with maleimidophenyl-polyethylene glycol facilitated by thiolation with iminothiolane: an approach to mask A, B, and D antigens to generate universal red blood cells.

Background: The surface decoration of red blood cells (RBCs) by polyethylene glycol (PEG) chains has been an approach developed to camouflage the blood group antigens from their antibodies. A PEGylation protocol, however, that can mask the antigens appropriately to inhibit the agglutination of RBCs with the respective antibodies is not available so far.

Study Design And Methods: A new approach for PEGylation of RBC membrane proteins has been designed with thiolation-mediated maleimide chemistry.

Successful rescue therapy with plasmapheresis and intravenous immunoglobulin for acute humoral renal transplant rejection.

Plasmapheresis (PP) and intravenous immunoglobulin (IVIg) remove donor-specific antibodies, a cause of acute humoral rejection (AHR). We describe the use of PP and IVIg as rescue therapy for AHR. The records of 143 renal transplants performed between October 1, 2000 and April 1, 2002 were reviewed.