The utility of therapeutic plasma exchange in Hyperviscosity syndrome associated with juvenile rheumatoid arthritis: A case report.

Hyperviscosity syndrome (HVS) is a life-threatening syndrome caused by high concentrations of large plasma proteins like IgM, rheumatoid factor, and other immune complexes, leading to increased blood viscosity and symptoms such as visual abnormalities, neurological impairment, bleeding diathesis, and thrombosis. While Waldenström's macroglobulinemia accounts for 80% to 90% of cases, HVS may develop in other clinical settings characterized by elevations in plasma proteins. Limited evidence currently exists describing the safety and efficacy of therapeutic plasma exchange (TPE) for the management of HVS secondary to non-neoplastic conditions. We report a case of recurrent HVS associated with juvenile rheumatoid arthritis and Felty syndrome that demonstrated improvement in clinical symptoms following initiation of TPE. These findings suggest that TPE may be utilized as an adjunct treatment option in patients with HVS secondary to autoimmune disorders.

Role of plasma exchange in stiff person syndrome.

OBJECTIVE: Stiff person syndrome (SPS) is commonly associated with antibodies directed against 65-kDa glutamic acid decarboxylase (GAD65). Therapeutic Plasma Exchange (TPE) has been used as an adjunct therapy in patients who do not respond well to conventional treatment, which includes immunosuppression therapies, anti-anxiety medications, muscle relaxants, anticonvulsants, and pain relievers. METHODS: We retrospectively analyzed the clinical data and outcomes of ten patients with the clinical diagnosis of anti-GAD65 positive SPS in which TPE was employed to improve symptoms refractory to conventional treatment during an eight-year period. RESULTS: TPE was initiated as complementary therapy in patients with worsening of symptoms characteristic of SPS. Six patients underwent chronic treatment with TPE following an initial course, of which the frequency of TPE was guided by the clinical response. Two patients only had transient improvements with further disease progression. Four patients developed a relapse of symptoms when the interval between procedures was increased. One of the four patients dependent on TPE had worsening of symptoms following complete cessation of TPE due to lack of insurance coverage. Four patients underwent only an acute hospitalized course of treatment with TPE; one demonstrated complete resolution of symptoms; one had a partial response; and two experienced no improvement. CONCLUSION: Our study supports previous reports that TPE may be beneficial for the management of patients with anti-GAD65 positive SPS, both for acute exacerbations and long-term maintenance, either as an adjunct therapy, or in lieu of treatment with disease modifying agents.

Advantages of isovolemic hemodilution-red cell exchange therapy to prevent recurrent stroke in sickle cell anemia patients.

Chronic simple hypertransfusion (every 3 to 4 weeks) effectively prevents secondary stroke in children with sickle cell anemia but leads to iron overload despite chelation therapy. Conventional red blood cell exchange (C-RBCx) has advantages over simple transfusion: no net iron gain and less frequent hospital visits. However, C-RBCx requires more red blood cell units, an apheresis instrument and skilled personnel; it is also more expensive. We developed a modified procedure where isovolemic hemodilution precedes RBCx (IHD-RBCx) to decrease RBC units required and to increase the interval between procedures. Twenty patients underwent IHD-RBCx over a period of 7 years. IHD-RBCx required 11% fewer RBC units and increased inter-procedure interval from 37 to 53 days compared to C-RBCx. The median number of annual procedures decreased from 9.8 to 7.0 per patient, resulting in estimated savings of more than $4.5 million over 10 years for 20 patients while providing improved care. Five patients have discontinued chelation therapy; three while on C-RBCx and two while on IHD-RBCx. No adverse events occurred related to the isovolemic hemodilution phase and no patients had recurrent stroke. IHD-RBCx is a safe, efficient, and cost effective therapy for secondary prevention of stroke in patients with sickle cell anemia.

Prospective monitoring of plasma and platelet transfusions in a large teaching hospital results in significant cost reduction.

BACKGROUND: Plasma and platelets (PLTs) are often transfused to correct mild to moderately abnormal laboratory values. Our objective was to reduce unnecessary plasma and PLT transfusions to nonbleeding patients by prospective triage and education of end users in evidence-based hemostasis and transfusion medicine practices. STUDY DESIGN AND METHODS: Using the Parkland Memorial Hospital's transfusion service and admission database as the data source, this study comprises the comparison of transfusion data on plasma and PLT use between pre- (2000-2002) and posttriage (2003-2006) periods. Yearly transfusion and wastage data on red blood cells (RBCs), plasma, and PLTs and yearly hospital admissions, trauma visits, and surgical procedures were extracted retrospectively for the study. RESULTS: The study revealed that implementation of triage resulted in a significant reduction of plasma (60%) and PLT (25%) transfusions, saving more than $3,000,000 over 4 years. CONCLUSIONS: Prospective triage and evidence-based transfusion practice education reduced unnecessary plasma and PLT transfusions and health care costs.

Whole blood in the management of hypovolemia due to obstetric hemorrhage.

OBJECTIVE: To study the use of blood products including whole blood, for the management of obstetric hemorrhage requiring transfusion. METHODS: This was a population-based, observational study of all women receiving blood for hypovolemia because of hemorrhage at the Parkland obstetrics service between March 24, 2002, and June 12, 2006. Hypovolemia was diagnosed in women who sustained hemorrhages sufficient enough to provoke hemodynamic instability. RESULTS: A total of 66,369 women gave birth during the study period, and 1,540 (2.3%) received a blood transfusion. Six hundred fifty-nine (43%) received only whole blood, 593 (39%) received only packed red blood cells, and 288 (19%) received combinations of blood products, including thawed plasma, platelets, and cryoprecipitate. The number of units transfused was similar in the whole blood and packed red blood cell groups (mean 2 units) and higher in the combination group (mean 5.5 units). Complications attributable to hypovolemia were similar in frequency in the whole blood and packed red blood cells groups, including intensive care unit admission (1%), hypofibrinogenemia (0.3%), and adult respiratory syndrome (0.5% compared with .3%). Acute tubular necrosis was more common in the packed red blood cell group (2% compared with 0.3%, P<.001). All of these outcomes were increased in the combination transfusion group. There were three maternal deaths in the cohort, two in the combination group and one in the packed red blood cells group. CONCLUSION: The risk of acute tubular necrosis is significantly reduced in women receiving whole blood transfusion for hypovolemia due to obstetric hemorrhage. LEVEL OF EVIDENCE: III.