Symptomatic Hyperviscosity Research Articles

Hyperviscosity syndrome secondary to a myeloma‐associated IgG1κ paraprotein strongly reactive against the HIV‐1 p24 gag antigen

Hyperviscosity syndrome secondary to hypergammaglobulinemia is a rare and potentially fatal complication in patients with human immunodeficiency virus type-1 (HIV-1) infection. We studied an HIV-1-positive patient with symptomatic hyperviscosity attributable to IgG1κ multiple myeloma. The patient initially responded to plasmapheresis and was subsequently treated with cytotoxic immunosuppressive chemotherapy. The patient remained asymptomatic during a 3-year follow-up period. The monoclonal IgG1κ gammopathy evolved to a biclonal variant of the same subtype with an expansion of marrow plasma cell population. Western blot analysis demonstrated that this myeloma-associated paraprotein was strongly reactive against the HIV-1 p24 gag antigen. Am. J. Hematol. 64:210–213, 2000. © 2000 Wiley-Liss, Inc.

Symptomatic hyperviscosity syndrome with polyclonal gammopathy responding to therapeutic plasmapheresis in a patient with myelodysplastic syndrome

Symptomatic hyperviscosity syndrome with polyclonal gammopathy responding to therapeutic plasmapheresis in a patient with myelodysplastic syndrome

Asymptomatic syndrome of polycythemic hyperviscosity: effect of partial plasma exchange transfusion

We determined the cerebral hemodynamic changes in infants with asymptomatic polycythemic hyperviscosity syndrome and whether treatment with partial plasma exchange transfusion (PPET) would affect hemodynamics as well as outcome. From a routine cord blood hematocrit screening, 71 babies were identified as needing to be tested for polycythemic hyperviscosity. In addition to clinical evaluation, each infant had radial artery hematocrit and viscosity determinations, blood gas determinations, cerebral blood flow velocity studies, cranial ultrasonography, and noninvasive intracranial pressure determination. Babies with symptomatic hyperviscosity (n = 17) were treated by PPET, whereas those with asymptomatic hyperviscosity (n = 28) were randomly selected to have PPET (n = 14) or to be observed (n = 14). The remaining babies (n = 26) with normal viscosity served as control subjects. Both hematocrit and viscosity decreased after PPET but remained unchanged in babies with hyperviscosity who were merely observed. Reversal of cerebral blood flow velocity abnormalities was observed after PPET in the infants with symptomatic hyperviscosity, whereas those who had no symptoms had normal results on Doppler studies at the outset, and no significant changes occurred with either PPET or observation. There were two deaths in the group with symptoms. A total of 46 babies returned for follow-up evaluation at a mean age of 30 +/- 7.7 months. Outcome of the control group was no better than that of those who had hyperviscosity, and outcomes did not differ between the babies with symptomatic and those with asymptomatic hyperviscosity, nor between those treated with PPET and those who were only observed. Multivariate analysis revealed that other perinatal risk factors and race rather than polycythemia or PPET, significantly influenced long-term outcome.

Waldenström's Macroglobulinemia: Long-Term Results with the M-2 Protocol

Thirty-three patients with symptomatic Waldenström's macroglobulinemia have been treated with the M-2 protocol (BCNU, cyclophosphamide, vincristine, melphalan, and prednisone). Therapy was administered every 5 weeks for 2 years and every 10 weeks for an additional 1-3 years. Median clinical and laboratory parameters included age 70 years (range 52-87), performance status 1 (1-3), prior therapy 7, weight loss 12, symptomatic hyperviscosity 13, splenomegaly 22, lymphadenopathy 7, hemoglobin 9.6 g/dl (6.7-14.6), IgM paraprotein level 2000 mg% (340-11,600), and serum viscosity 2.1 (1.4-6.0). Responses were observed in 27 patients, of whom 21 were partial responses. Survival ranges from 1 to 120+ months with 58% of patients projected to be alive at 10 years. Twenty-one patients remain alive, of whom 10 are greater than or equal to 6 years from initiation of therapy with M-2. Treatment has been well tolerated with usually only mild to moderate hematologic toxicity. Median nadir white blood cells during the first cycle was 3000/mm3 (1000-5500). Peripheral neuropathy was seen in 54% secondary to vincristine. Nausea/vomiting, anemia requiring transfusions, and alopecia were each noted in approximately 25% of patients. Sepsis was observed in 2 patients. Two characteristics, age and prior therapy, were found to be of borderline statistical significance (p = 0.03) using univariate analysis but were not significant with multivariate analysis. The M-2 protocol may be able to produce prolonged survival in the majority of patients with Waldenström's macroglobulinemia. Additional trials are needed to develop recommendations for therapy as well as factors predictive for survival and suitability for treatment.

Adults with cyanotic congenital heart disease: hematologic management.

Hematologic management of adults with cyanotic congenital heart disease has received little recent attention. The lack of practical therapeutic guidelines prompted us to consolidate our observations on 124 cyanotic adults for general physicians, cardiologists, and hematologists who care for these patients. Specific attention focused on regulation of erythrocyte mass and concepts of compensated and decompensated erythrocytosis, symptoms of deficient tissue oxygen transport, hyperviscosity and iron deficiency, the potential relation between elevated hematocrit levels and brain injury, hemostasis, urate metabolism, and renal function. Cerebral infarction was not seen in any patient. Phlebotomy is best reserved for treatment of symptomatic hyperviscosity. Iron therapy is indicated for symptomatic iron deficient erythropoiesis. Abnormal hemostatic mechanisms are the rule. Antithrombotic medications have little or no role in treatment. Hyperuricemia is the result of abnormal renal uric acid excretion not urate overproduction, and serves as a marker of abnormal renal function. Drugs that promote urate excretion are the preferred maintenance treatment in symptomatic hyperuricemic patients.

WHOLE BLOOD VISCOSITY DETERMINED BY PLASMA PROTEIN, HEMATOCRT, MEAN CORPUSCULAR VOLUME

Therapeutic decisions in the management of neonatal hyperviscosity are often made on the basis of hematocrit (Hct) values only. However, in tubes with the diameter of arterioles, whole blood viscosity (WBV) increases also with plasma viscosity (PV), red blood cell (RBC) aggregation and impaired RBC deformability. In order to determine the correlation between total plasma protein (TPP) as a measure of PV, and Hct at various mean corpuscular volumes (MCV) on a WBV of 4.2cP previously found to be clinically significant, we adjusted the placental blood of 20 premature and 10 term infants, and the venous blood of 10 adults to a Hct between 50 and 70% and measured TPP and WBV (100μm-capillary viscometer). From these data a nomogram for WBV = 4.2cP was constructed. Thus, a plot for TPP and Hct below the appropriate MCV line indicates a WBV with a lower risk for symptomatic hyperviscosity. The regression equation expresses the critical Hct for this WBV as it is influenced by TPP and MCV. It will be noted that certain therapeutic measures e.g., transfusions with plasma, albumin, or RBC with their attendant change in TPP and MCV may alter whole blood viscosity unfavorably.

Combination Chemotherapy (M-2) Protocol (BCNU, Cyclophosphamide, Vincristine, Melphalan, and Prednisone) for Waldenström's Macroglobulinemia: Preliminary Report

Fourteen consecutively referred, symptomatic patients with Waldenström's macroglobulinemia (ages 52-87 yr) have been treated with the 5-drug M-2 protocol (BCNU, cyclophosphamide, vincristine, melphalan, and prednisone). Three patients were previously treated and 11 patients were untreated. The majority of patients were symptomatic from hyperviscosity. All patients have responded to therapy. Two patients have achieved complete remissions and 12 patients partial remissions to date. None of the patients with symptomatic hyperviscosity has required plasmapheresis since therapy with the M-2 has been initiated. Lymphadenopathy, hepatosplenomegaly, and anemia have also responded to treatment. Follow-up data are limited, with survival from initiation of therapy with the M-2 ranging from 2+ to 35% mo (median 17+ mo) 2+-40+ mo from time of diagnosis). Combination chemotherapy for Waldenström's macroglobulinemia with the M-2 protocol appears to increase the response rate in patients with symptomatic disease. Further survival analysis will be carried out.

Combination chemotherapy (M-2) protocol (BCNU, cyclophosphamide, vincristine, melphalan, and prednisone) for Waldenstrom macroglobulinemia: preliminary report

Fourteen consecutively referred, symptomatic patients with Waldenstrom's macroglobulinemia (ages 52–87 yr) have been treated with the 5-drug M-2 protocol (BCNU, cyclophosphamide, vincristine, melphalan, and prednisone). Three patients were previously treated and 11 patients were untreated. The majority of patients were symptomatic from hyperviscosity. All patients have responded to therapy. Two patients have achieved complete remissions and 12 patients partial remissions to date. None of the patients with symptomatic hyperviscosity has required plasmapheresis since therapy with the M-2 has been initiated. Lymphadenopathy, hepatosplenomegaly, and anemia have also responded to treatment. Follow-up data are limited, with survival from initiation of therapy with the M-2 ranging from 2+ to 35% mo (median 17+ mo) 2+-40+ mo from time of diagnosis). Combination chemotherapy for Waldenstrom's macroglobulinemia with the M-2 protocol appears to increase the response rate in patients with symptomatic disease. Further survival analysis will be carried out.

Hyperviscosity in congenital heart disease

Viscosity or blood "fluidity" has been studied in relation to hematocrit in normal reconstituted human blood and in blood from children with congenital heart disease. At 37°C. hematocrit and viscosity are consistently related, but at higher hematocrits above 60 per cent, small increments in hematocrit produce large increments in viscosity. At low shear rates, such as exist in small blood vessels, blood is less fluid at high hematocrits; this becomes critical above hematocrits of 70 per cent. An example of a patient with extreme and symptomatic hyperviscosity and evidence of intravascular coagulation due to cyanotic congenital heart disease was given. This patient was treated by phlebotomy, which was associated with improvement evident clinically and in laboratory data. These studies indicate that hyperviscosity occurs in polycythemic cyanotic congenital heart disease. Measurements of blood viscosity provide objective information of value in determining the advisability of phlebotomy in selected cases.