Quantification of α‐Chain Excess in Erythrocytes in β‐Thalassaemia by Microinterferometry

Summary: Microinterferometry was used to determine the dry mass of α‐chain precipitates in erythrocytes from four patients with homozygous β‐thalassaemia. α‐Chain precipitates represented on the average 40% of the total dry mass of cells with measurable amounts of precipitation. The maximum load of α‐chain inclusions in a single cell was 75% of the dry mass. A linear correlation was established between the amount of total haemoglobin and non‐α‐chains in individual cells, and the amount of α‐chain precipitation increased linearly with decreasing amounts of soluble haemoglobin, thus indicating an association between impaired haemoglobin production and lack of non‐α‐chains at undisturbed α‐chain production. The mean ratio of the quantity of α/(β+γ+δ) chain in individual patients varied between 2.3 and 3.0. These ratios are lower than expected from synthetic rate studies, thus indicating the possibility of some precipitate degradation in vivo. Copyright © 1972, Wiley Blackwell. All rights reserve

Quantitation of hemoglobin in single erythrocytes with and without fetal hemoglobin

The hemoglobin content was determined by microspectrophotometry in single erythrocytes with and without fetal hemoglobin (Hb F) from 16 normal subjects, 30 patients with anemia of different etiology and severity and 20 individuals with thalassemic disorders. Hb F‐containing cells were identified by an indirect immunofluorescent method. The relative single cell value: total extinction (TE) at 415 nm, cell size (A) and the ratio TE/A, were used to indicate single cell values of MCH, MCV and MCHC respectively. The TE (MCH) and/or TE/A (MCHC) did not differ significantly between Hb F‐containing (F‐cells) and non‐F cells in normal subjects and in cases with various forms of acquired anemia. On the contrary, the TE and/or TE/A of F‐cells was found significantly higher in F compared to non‐F cells in 11 of 20 (55%) of the cases with thalassemia. The results suggest that, in some cases of thalassemia, hemoglobin F is an important substitute for hemoglobin A and may improve the level of hemoglobinization. © Munksgaard 198

Evidence for sinoatrial blockade associated with high dose cytarabine therapy

Cytarabine therapy is rarely complicated by cardiotoxicity. The present report describes the clinical course of a 35-year-old female patient with acute myelogenous leukemia in complete remission, who developed sinus bradycardia while on high dose cytarabine as a consolidation therapy. The electrocardiographic findings suggested that bradycardia was most probably the result of sinoatrial blockade. The available information regarding a possible association of cytarabine with disturbances of cardiac rhythm is reviewed. (C) 1998 Elsevier Science Ltd. All rights reserved

RANDOMIZED CLINICAL-TRIAL COMPARING CEFTRIAXONE AMIKACIN VERSUS CEFTAZIDIME AMIKACIN AS INITIAL THERAPY OF FEBRILE EPISODES IN NEUTROPENIC PATIENTS

Neutropenic patients with underlying hematologic (usually malignant) diseases were randomized to receive either 2 g ceftriaxone once daily + 0.5 g amikacin or 2 g ceftazidime twice daily + 0.5 g amikacin b.i.d. when fever was higher than 38-degrees-C and granulocyte counts less than 0.5 x 10(9)/l. 25 patients were included in each treatment group. Successful outcome of treatment was observed in 28 (13/15) and in an additional 5 (2/3) patients after modification of the therapy. Tolerability was excellent in both groups

Acute promyelocytic leukemia relapsing into FAB-M2 acute myeloid leukemia with trisomy 8

Acute promyelocytic leukemia was diagnosed in a 48-year-old man; the karyotype was normal, whereas reverse transcriptase polymerase chain reaction (RT-PCR) analysis identified PML/RAR alpha chimeric transcripts of the bcr3 type. Rather unexpectedly, the patient did not respond to all-trans retinoic acid administration; he attained complete remission with conventional chemotherapy and became PML/RAR alpha negative. Two years later while PML/RAR alpha negative nn RT-PCR he presented with thrombocytopenia. Bone marrow examination was compatible with myelodysplasia of the RAEB type; the karyotype was normal. Then, after 10 months, he developed overt acute myeloid leukemia with PML/RAR alpha negative, French-American-British MZ blasts; karyotypic analysis revealed mosaicism for trisomy 8. (C) Elsevier Science Inc., 2000. All rights reserved