Síndrome de hiperviscosidad en pacientes con mieloma múltiple / Hyperviscosity syndrome in patients with multiple myeloma

Objetivos: el mieloma múltiple es una enfermedad de distribución universal. Se determinaron la frecuencia, las características y el tratamiento del síndrome de hiperviscosidad como complicación de esta enfermedad. Método: se realizó un estudio descriptivo retrospectivo que incluyó a 31 pacientes con el diagnóstico de mieloma múltiple atendidos en el servicio de adultos del Instituto de Hematología e Inmunología desde enero del 2007 a junio del 2011. Resultados: la edad media fue de 61 años con un predominio del sexo masculino y el color de la piel blanca. Los pacientes se presentaron con anemia, eritrosedimentación acelerada, hiperproteinemia, hipoalbuminemia, hipergammaglobulinemia, una infiltración media de la médula ósea por células plasmáticas de 63.2 porciento y una viscosidad sérica media de 2.93. El síndrome de hiperviscosidad se observó en 4 (12.9 porciento) pacientes. Las manifestaciones clínicas principales del síndrome de hiperviscosidad fueron los síntomas generales y los síntomas neurológicos y se presentaron con cifras de viscosidad sérica iguales o superiores a 4.5. La proteína monoclonal identificada como responsable del síndrome de hiperviscosidad fue la IgA y la viscosidad sérica fue proporcional a las concentraciones de la paraproteína (p<0.01). Conclusión: el síndrome de hiperviscosidad se presentó en los pacientes estudiados con similares características a lo reportado por otros autores. La plasmaféresis y la quimioterapia fueron los pilares del tratamiento con resultados satisfactorios

Objectives: multiple myeloma is a disease of worldwide distribution. We determined frequency, characteristics and treatment of hyperviscosity syndrome as a complication of this condition. Method: we conducted a retrospective study involving 31 patients with a diagnosis of multiple myeloma treated at the Adult Hematology Service of the Institute of Hematology and Immunology from January 2007 to June 2011. Results: mean age was 61 years with a male predominance and slight predominance in caucasians. The patients presented with anemia, high erythrosedimentation rate, hyperproteinemia, hypoalbuminemia, hypergammaglobulinemia, a median infiltration of bone marrow by plasma cells of 63.2percent and an average serum viscosity of 2.93. Hyperviscosity syndrome was observed in 4 (12.9 percent) patients. The main clinical manifestations of hyperviscosity syndrome were general symptoms and neurological symptoms and presented with serum viscosity at or above 4.5. The monoclonal protein identified as the responsible of the hyperviscosity syndrome was IgA and viscosity was proportional to the concentrations of the paraprotein (p <0.01). Conclusion: the hyperviscosity syndrome is presented in the studied patients with similar characteristics to those reported by other authors. Plasmapheresis and chemotherapy were the mainstays of treatment with satisfactory results

Utilization of Post-centrifugal Plasma Filtration for Treatment of Hyperviscosity Syndrome due to Waldenstrom's Macroglobulinemia: A Case Report / 대한수혈학회지

Waldenstrom's macroglobulinemia is characterized by overproduction of immunoglobulin M (IgM), which can lead to development of a hyperviscosity syndrome. Plasmapheresis has been used to reduce plasma viscosity and IgM levels. However, plasmapheresis not only removes pathogenic substances but also the normal components in plasma. Post-centrifugal plasma filtration (PCPF) using a filter with an appropriate pore size has been shown to be effective for selective removal of macromolecules in plasma without requiring a replacement fluid. Here, we report on a patient with hyperviscosity syndrome due to Waldenstrom's macroglobulinemia, which was ameliorated by a single treatment of PCPF using an Evaflux 5A filter. After centrifugation, the separated plasma of the patient was subjected to membrane filtration for removal of IgM in the plasma. After PCPF, a 56.5% reduction in the IgM level (from 9,370 mg/dL to 4,080 mg/dL) was achieved, whereas the level of albumin showed a slight decrease, from 2.7 g/dL to 2.4 g/dL (11.1% reduction). The patient's symptoms were relieved and she was discharged on the seventh hospital day.

A Case of Waldenstrom's Macroglobulinemia Treated with 2-Chlorodeoxyadenosine (Cladribine) / 대한혈액학회지

Waldenstrom's macroglobulinemia is a low- grade lymphoproliferative disorder with monoclonal IgM protein. It is characterized by normocytic, normochromic anemia and lymphoplasmacytic marrow infiltration. Chemotherapy with alkylating agents and steroids has been the standard therapy for patients with symptomatic macroglobulinemia. The purine nucleoside analogues, either alone or in combination with other chemotherapeutic agents are increasingly used, and approximately 40% of patients who have received prior therapy with alkylating agents responded. We experienced a case of Waldenstrom's macroglobulinemia suc-cessfully treated with three courses of cladribine, who had previously received unsuccessful therapy using an alkylating agent, steroid and plasmapheresis. Treatment was well tolerated except for frequent upper respiratory infections with severe pancytopenia. A marked and sustained bone marrow suppression occurred in this patient but resolved in three months without any severe infection.

Sudden Hearing Loss in Chronic Myelogenous Leukemia Implicating Hyperviscosity Syndrome / 대한이비인후과학회지

Sudden sensorineural hearing loss due to the initial manifestation of hematologic disease is very rare. Chronic myelogenous leukemia has been implicated as a causative factor of sudden sensorineural hearing loss. Leukemic infiltration, hemorrhage, infection, and hyperviscosity have been suggested as possible mechanisms in patients with chronic myelogenous leukemia. A 49-year-old male presented unilateral sudden sensorineural hearing loss. The patient was found to have chronic myelogenous leukemia during the work-up for the hearing loss. The WBC count upon admission was 485,100/mm(3). Hemoglobin, hematocrit, and platelet count were within the normal limits. The patient underwent three cycles of leukapheresis and chemotherapy with interferon alpha and hydroxyurea for the treatment of leukemia. The hearing threshold level was 75 dB on admission. It improved to 35 dB when the WBC count fell to 294,000/mm(3), and finally settled at 32 dB two weeks after the termination of chemotherapy when the WBC count was 125,900/mm(3). We present a case of a chronic myelogenous leukemia patient who initially presented with sudden sensorineural hearing loss. We presume that cochlear vessel occlusion as a result of elevated blood viscosity was responsible for this patient's hearing loss. Early onset of sudden deafness in a chronic myelogenous leukemia patient may be due to the hyperviscosity syndrome and be possible to reverse hearing loss through early leukapheresis.

Central Retinal Vein Occlusion in Multiple Myeloma Associated with Hyperviscosity Syndrome

Multiple myeloma is a malignant proliferation of the atypical plasma cells which usually involves antibody synthesis in the immune system. Systemic manifestations include anemia, renal failure, osteoporosis, immune deficiency symptom. Ophthalmolgically this disease involves cornea, conjunctiva, uvea, iris, ciliary body, retinal vessels, extraocular muscles and optic nerve. The authors experienced a case of multiple myeloma with hyperviscosity syndrome who presented with central retinal vein occlusion in both eye.

Hyperviscosity Syndrome in Newborn

We experienced Hyperviscosity syndrome in 5 newborn infants during 6 months period from August 1980 to January 1981. Diagnosis was made on the basis of characteristic clinical symp-toms along with polythemia. The following results were obtained. Sex in 5 affected infants showed male in 2 and female in 3. And gestational age showed preterm in 3 and full term in 2 cases. Predisposing factors were intertwin transfusion in 1, placental insufficiency with maternal to-xemia in 2(one of which was accompained with interwin transfusion) and small for gestational age in 1. Signs and symptoms associated with hyperviscosity syndrome were cyanosis in 3, dyspnea in 2, lethargy in 2, plethora in 1, hypocalcemia in 4, hypoglycemia in 2 and hyperbilirubinemia in 2 cases. Partial exchange transfusion was done in all cases, resulting in improvement of polycythem-ia and clinical condition, except 1 expired case.

Correlation between expression of platelet membrane glycoproteins and hyperviscosity syndrome / 第二军医大学学报

Objective:To study the relationship between hyperviscosity syndrome(HVS) and platelet membrane glycoproteins(GP) sub group changes.Methods: CD62P,CD63,CD31 and CD41 were measured by flow cytometry(FCM). The hemorrheoloic parameters of HVS patients were compared with those of normal aged group and normal young group.Results:(1)The CD62P,CD63 and CD31 of aged and young HVS patients before therapy were obviously higher than those of the normal aged group and normal young group.(2)Combined measurement could improve the detecting positive rate of HVS patients up to 92% 100%, with the sensitivity being 95.3%.(3)Bamyl decreased CD62P,CD63 and CD31,which was obviously correlated with ?b and ?p. Conclusion:The alteration of platelet membrane GP positive expression may be a parameter for early diagnosis and clinical outcome observation.