Clinical Features of Waldenstrom Macroglobulinemia in Korea

BACKGROUND: Waldenstrom macroglobulinemia (WM) is a lymphoproliferative disorder characterized by monoclonal IgM. Its rarity makes it difficult to know the clinical manifestations and outcomes of patients with WM. METHODS: The clinical records of 13 patients diagnosed with WM between 1983 and 2003 were reviewed, and 12 patients were eligible. RESULTS: The median age was 57 years (range, 40 to 85), and the male to female ratio was 2. B symptoms and hyperviscosity requiring plasmapheresis existed in 5 and 4 patients, respectively, at the time of diagnosis. Hepatomegaly and splenomegaly were detected in 5 and 3 patients, respectively. Sites of extranodal involvement were bone (3) and lung (1) in 3 patients. The peripheral neuropathy was complicated in 3 patients. (Ed note: check this sentence.) Cryoglobulin was checked in 6 patients and it was detected in 3 of them. The median concentration of serum IgM was 4.2 g/dL (0.7~6.2). The median albumin, hemoglobin, WBC, and platelet levels were 2.8 g/dL, 8 g/dL, 5, 400/micro L, and 138, 000/micro L, respectively. One patient had transitional cell carcinoma concomitantly, and one patient developed small cell lung cancer. Of the 11 patients receiving chemotherapy (7-chlorambucil, 2-melphalan, 1-cyclophosphamide, 1-CHOP), 4 patients showed the objective responses including 2 complete remissions, but they all ultimately relapsed. The response rate of second-line therapy was 14% (1/7). After a median follow-up of 20 months, 3 patients were still alive with disease. The median overall and progression-free survival were 24 months (95% confidence interval (CI) : 5-43) and 24 months (95% CI: 8-40), respectively. CONCLUSION: The initial high levels of serum IgM and severe anemia reflect a lack of suspicion of WM at the early stage. Careful suspicion and proper diagnostic approaches will allow more patients to show an improved outcome.

Paraproteins: a regional South Australian experience.

We have performed a systematic review of all new serum and urinary paraproteins detected over a six year period in an immunodiagnostic laboratory serving a population of 400,000 people. Clinical diagnoses and associated laboratory features were ascertained from a computerized laboratory database or from clinical notes. Over the period of study, serum or urine paraproteins were detected in 613 new patients. These consisted of 568 patients with serum paraproteins and 45 patients with urinary monoclonal free light chain (in the absence of a serum paraprotein). These paraproteins occurred more commonly in males and the frequency increased with age. Approximately 30% of the serum paraproteins and 60% of urinary monoclonal free light chain were associated with B cell lymphoproliferative disorders (multiple myeloma, plasmacytoma, Waldenstrom's macroglobulinemia, non-Hodgkins lymphoma, chronic lymphocytic leukemia, etc) with the remainder being labeled as monoclonal gammopathies of uncertain significance (MGUS). At clinical presentation, patients with lymphoproliferative disorders tended to have higher levels of paraprotein, B2 microglobulin, the presence of free urinary light chain and demonstrated molecular size heterogeneity of the paraprotein but there was considerable overlap. A good correlation was noted between paraprotein concentration and viscosity in most patients. In conclusion paraproteins were most frequently encountered in the context of a gammopathy of uncertain significance. Features which suggested lymphoproliferative disorders included higher levels of serum paraprotein (> 15 g/l), elevated levels of B2-microglobulin and the presence of urinary free high chain. However, as much overlap was seen with patients with MGUS, regular monitoring of paraprotein level is considered mandatory in the management of these patients.

Haemorheological behaviour of erythrocytes & risk factor profile in patients suffering from Waldenström's macroglobulinaemia.

The plasma viscosity, haematocrit, and erythrocyte aggregation and deformability of blood samples of patients of Waldenström's macroglobulinaemia were measured by capillary viscometer, microcentrifuge, Myrenne aggregometer and filtrometer respectively and correlated with the serum IgM concentrations. The observed increase in plasma viscosity and erythrocyte aggregation and decrease in their deformability were attributed to the increase in the concentration of IgM. Haematocrit was also decreased compared to normal subjects. Based on these parameters a haemorheological risk factor profile was developed and an overall risk factor calculated. An increase in the risk factor in these patients was observed. The haemorheological profile and the calculated risk factors not only help in establishing the inter-relationship of these parameters in the disease state but also to evaluate the severity of the disease.

Viscosidad sanguínea humana implementación de un método sencillo para sangre total y plasma valores normales / Human blood viscosity use of a simple method for total blood and plasma normal values

Se implementa una técnica sencilla para medir la viscosidad sanguínea y plasmática, midiendo el tiempo que demora una cantidad determinada de sangre o plasma en recorrer un segmento de tubo, a temperatura y presión constante. Se analizan 100 muestras de sujetos normales, de acuerdo a parámetros determinados al inicio del estudio. Se concluyen valores expresados en viscosidad relativa similares a los encontrados en viscosímetros por otros autores. Promedio 4,5 u de viscosidad sanguínea para hombres y mujeres, y 1,9 u de viscosidad plasmática en mujeres y hombres, respectivamente. De esto concluimos que no hay diferencias significativas entre viscosidad sanguínea y viscosidad plasmática según sexo, edad, hematocrito normal y el tiempo transcurrido entre la toma de muestra y la medición en laboratorio para el grupo en estudio. Se deja planteada la inquietud de continuar analizando la viscosidad sanguínea de acuerdo a otros parámetros, como diabetes descompensada, accidentes vasculares u otras patologías o cuadros clínicos en que se describen alteraciones de viscosidad sanguínea

Lupus anticoagulant and Waldenstrom's macroglobulinemia

Una prolongación de las pruebas de coagulación dependientes de fosfolípidos fue detectada en una paciente de 62 años, con una dermatitis linfocítica, pero que no tenía problemas de sangría ni trombóticos. Se detectó una gamapatía monoclonal de tipo IgM lambda con hipoglobulinemia IgG e IgA. La sangre periférica y la médula ósea eran normales inicialmente. La serología fue falsa positiva confirmada por la prueba de anticardiolipina. Todos los estudios para enfermedades infecciosas o autoinmunes fueron negativos. La paciente desarrolló fiebre y esplenomegalia por lo que se le practicó una explenectomía. La histología demostró un neoplasma plasmático. La inmunoperoxidase demostró cadenas lambda en las células anormales y la microscopía electrónica confirmó el origen plasmático del tumor. La citometría de flujo en sangre periférica demostró la mayor parte de células IgM y con marcadores lambda. La coagulación demostró la prolongación del PT y PTT aún con mezcla de plasma normal. La prueba de inhibición de tromboplastina de tejido fue anormal. La prueba de neutralización de plaquetas demostró corrección cuando se usó plaquetas en vez de fosfolípidos como reactivo, confirmando la presencia del anticoagulante de lupus. El lugar de producción de la proteína anormal fue confirmado por estudios inmunohistoquímicos. El anticoagulante de lupus fue neutralizado con un antisuero anti IgM confirmando la naturaleza del anticuerpo. El anticoagulante de lupus se ha descrito en varias enfemedades especialmente el lupus eritematoso, pero también en otras enfermedades autoinmunes, uso de medicamentos, enfermedades neoplásicas, abortos recurrentes, y el síndrome de inmunodeficiencia adquirida. Nuestro paciente tenía un desorden linfoplasmático produciendo hiperglobulinemia M que a su vez era el anticoagulante de lupus