The first report of myeloma with IgD κ and free κ in Indonesia.

IgD κ myeloma is a rare plasma cell neoplasm case and has never been reported before in Indonesia. In normal condition, IgD level in blood is very low, therefore increase of IgD level in myeloma could be missed by serum protein electrophoresis. A case of a 59 years old female with severe bone pain is reported. In radiology evaluation, there were thoracal compression fracture and thoracal foramen narrowing. For this patient, the myeloma diagnosis was based on WHO criteria, the stage IIIb was based on Durie and Salmon criteria, and bad prognosis with prognostic index stage III diagnosis was based on International Prognostic Index from International Myeloma Working Group, respectively. In serum protein electrophoresis we found a very small monoclonal spike and in immunofi xation there were monoclonal IgD κ and free light chain κ.

Peripheral blood and hemoglobin electrophoresis pattern in beta thalassemia major patients receiving repeated blood transfusion.

One hundred and fifteen beta thalassemia major outpatients attending the Thalassemia Center Department of Child Health, Medical School University of Indonesia Dr. Cipto Mangunkusumo General Hospital for routine blood transfusion and hematology examination, participated in this study. There was a negative correlation between the size of the spleen and the peripheral blood parameters. All peripheral blood parameters tend to decrease with the enlargement of the spleen, and the condition is reversed after splenectomy. We observed that hypersplenism starts when the spleen is as big as S (V – VI). The hemoglobin electrophoresis pattern from beta thalassemia major patients receiving repeated blood transfusion did not show a dense HbF fraction, 90 patients showed a normal hemoglobin electrophoresis pattern. A hemoglobin analysis of both parents could be useful to confirm the diagnosis of beta thalassemia major for patients receiving repeated blood transfusion. In order to get a definite diagnosis, a genetic analysis by bio molecular technique is needed.

Renal impairment in  thalassemia major patients receiving repeated blood transfusion.

β-thalassemia major is a disease caused by β polypeptide chain synthesis disorder which is inherited in an autosomal recessive manner from both parents and which is marked by little or no β-globin chain synthesis. Treatment for β-thalassemia major patients is by giving repeated blood transfusions, which causes iron accumulation, leading to hemochromatosis. Iron accumulation can occur in various body organ, including the kidneys. The aim of this study was to investigate the existence of renal impairment in β-thalassemia major patients. The subjects of this study were β-thalassemia major patients aged 15 - 28 years old who had received 6 units of packed red cells or more within 6 months. In this study, urine and serum samples of the subjects were taken and examined. Assay of serum iron was performed with Hitachi 737. Results were that 94.7% patients showed an increase in transferrin saturation and 40% of them had hemochromatosis; 73.4% had microalbuminuria; 1.3% had albuminuria and 21.3% had increased urinary β2- microglobulin (β2-m). A total of 78.6% of patients showed renal impairment. Conclusion of this study suggested that glomerular dysfunction happens in an earlier stage of the disease process. The high incidence of microalbuminuria is also attributed to defective ability of the proximal tubular cells to reabsorb protein besides dysfunction of the glomeruli.

Rhabdomyolysis in Indonesian marathon athlete.

Serum and urine myoglobin assessments were conducted on 37 national marathon athletes who participated in the Asian Marathon III & Proklamaton XV. Three athletes showed a myoglobin value of < 50 ug/L and the other 34 athletes showed a myoglobin value of 211-3300 ug/L. Rhabdomyolysis were only found in 2 athletes, thus the prevalence of rhabdomyolysis is 6.1%. A correlation was found between myoglobinemia value and the athlete’s performance.

Diabetes mellitus in ß-thalassemia major patients.

β-thalassemia major is a disease caused by β polypeptide chain synthesis disorder which is inherited as an autosomal recessive from both parents which is marked by little or no β globin chain synthesis. Medication for β thalassemia major patients is by repeated blood transfusions, which causes hemochromatosis. Hemochromatosis can occur in various organs including the pancreas. The aim of the study was to assess the alteration of plasma glucose concentration and the hemochromatosis prevalence. Fasting plasma glucose concentration and serum ferritin examination were measured in 115 β thalassemia major patients with ages between 10-23 years who were out-patients in the Thalassemia Centre, Department of Child Health, Medical School, University of Indonesia / Dr. Cipto Mangunkusumo General Hospital, Jakarta. The plasma glucose concentration examination was conducted by the GDH enzymatic method, with American Diabetes Association (ADA) criteria in the evaluation, while the serum ferritin examination was conducted with the microparticle enzyme immuno assay (MEIA) method. All patients had hemochromatosis, 14.8% of the patients had impaired fasting glucose level and 2.6% of the patients showed indications of diabetes mellitus. β thalassemia major patients who receive frequent transfusions will develop hemochromatosis that will in turn impair the pancreatic function.

Evaluation of the stability of Cell Dyn 16 Tri Level as a control material for laboratory external quality assessment scheme on hematology.

The implementation of a laboratory test should always implement a laboratory quality control program, i.e internal quality control and external quality assessment. In an external quality assessment scheme, a control material that is stable over delivery until tested by the participating laboratory. In this study, we evaluated the stability of Cell Dyn 16 Tri Level (TL) control material at room temperature (26-32 degrees C), stored in a transport vessel containing ice pack, and the precision and accuracy of the instrument Cell Dyn 1400. The control used was Cell Dyn 16 TL with low value (L), normal value (N) and high value (H). This study was done in the Clinical Pathology Department of FKUI-RSCM during February 2001 until May 2001. Control material was stored room in a transport vessel containing ice pack for 15 days, then analysed macroscopically, microscopically and evaluated for its stability. Test for precision and accuracy was done within run and for precision between day on Cell Dyn 1400. The result of this study showed a macroscopic change beginning on day 14 (L) day 12 (N) and day 15 (H). Microscopic change was observed on day 13 (L and N) and day 15 (H), Erythrocyte and hemoglobin level was stable until day 15. Changes in leukocyte was seen on day 14 (L), day 12 (N) and day 15 (H). Platelet showed instability on day 9 (L), day 10 (N and H). Mean erythrocyte volume was out of range on day 15 (L), but the N and H control was still stable. The precision and accuracy of Cell Dyn 1400 was in WHO recommended range. We concluded that the precision and accuracy of Cell Dyn 1400 is good. Cell Dyn 16 TL control material was stable until day 9, and its can be recommended to be used as a control material for external quality assessment scheme.