摘要:

AbstractOver a 3‐year period, 26 patients with Philadelphia chromosome‐positive chronic granulocytic leukemia were studied cytogenetically in both the chronic and blastic transformation phases of the disease; a further three patients were studied only after blastic transformation. Sixteen were considered to have adequate evidence of the type of transformation and form the basis of this report, where chromosome changes have been correlated with the morphological type of blastic transformation.Seven patients developed a myeloblastic transformation, seven a lymphoblastic transformation, and two an erythroblastic transformation. All patients in the myeloid group acquired one or more of the nonrandom changes associated with CGL blastic transformation, viz. +8,i(17q), + 19,+22q‐. Patients in the lymphoblastic group acquired structural abnormalities, apparently random in nature and usually in a small percentage of cells. The two patients with erythroblastic transformation developed markedly hyperdiploid cells (>50 chromosomes) with both numerical and structural abnormalities. Patients in the lymphoblastic group appeared to have a slightly better prognosis than the myeloid group, whilst the patients with erythroblastic transformation had a very poor prog

ISSN:0361-8609    

DOI:10.1002/ajh.2830200402

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractA marked increase in calcium (Ca) uptake (85.5 ± 39.0 nmoles/ml RBC/2 hr) was observed in ATP‐depleted red cells of patients with paroxysmal nocturnal hemoglo‐binuria (PNH) as compared to ATP‐depleted normal red cells (8.0 ± 2.0). The extent of increased Ca uptake in the PNH red cells was related to the extent of increased sensitivity to complement, as judged by the sugar water test (p<0.001) and the acidified serum test (p<0.001), and inversely correlated to the decreased activity of red cell acetylcholinesterase (p<0.01). The results may indicate that increased calcium uptake in PNH red cells is a possible intrinsic defect of the red cell membranes in the d

ISSN:0361-8609    

DOI:10.1002/ajh.2830200403

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractHemolysis in paroxysmal nocturnal hemoglobinuria (PNH) is considered to be a result of an intrinsic membrane defect. This defect may result in abnormal material properties of PNH erythrocytes. To examine this hypothesis, fragmentation failure, and membrane deformability were assessed in the absence of complement by micropipette techniques. Membrane viscosity was determined by observing relaxation of deformed cells. Results show a bimodal distribution of force for membrane failure, membrane viscoelasticity, and elastic shear modulus. One population requires significantly less force for fragmentation, mean 0.56 × 10−6dyne; has increased membrane viscosity, mean 0.205 × 10−2dyne sec/cm; and has decreased elastic shear modulus, mean 0.56 × 10−2dyne/cm. A second population resembles control with fragmentation force, mean 1.19 × 10−6dyne, control 1.05 × 10−6dyne; membrane viscosity, mean 0.112 × 10−2dyne/cm, control 0.102 × 10−2dyne sec/cm; elastic shear modulus, mean 0.70 × 10−2dyne/cm, control 0.78 × 10−2dyne/cm. The percent of cells with abnormal material properties corresponds to the percent of PNH III cells determined by complement lysis. Thus, the hemolysis attributed to an abnormal clone of erythrocytes in PNH is associated with an intrinsic membrane abnormality

ISSN:0361-8609    

DOI:10.1002/ajh.2830200404

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractSerum beta2microglobulin levels (B2M) were evaluated before and during chemotherapy in 97 previously untreated patients with multiple myeloma. Pretreatment values were useful in confirming tumor mass grade, and marked reductions following chemotherapy correlated well with the onset of remission. No gain was evident from correcting the B2M for the level of serum creatinine. A pretreatment B2M value greater than 6 mg/L correlated with a low response rate and was the most important variable that predicted a short survival time.

ISSN:0361-8609    

DOI:10.1002/ajh.2830200405

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractRecent observations that the sickle RBC are excessively susceptible to phagocytosis by macrophages in vitro prompted me to look for evidence of in vivo erythrophagocytosis (Ep) in patients with sickle cell anemia (SS). Freshly prepared smears of unmanipulated blood of 27 patients with SS in steady state were examined for Ep by a 500‐cell differential white blood cell (WBC) count performed in duplicate. Ten of 27 (37%) SS patients showed Ep (1–6/1,000 WBC or 1–10/100 monocytes). By contrast, no Ep was found in similarly prepared blood smears of 25 normal adult controls and nine splenectomized subjects. The mean hemotocrit value of the Ep(+) SS patients was significantly lower than that of the Ep(−) patients (21.0 ± 1.7% vs 24.0 ± 2.7% p<0.01). Considering the rarity of spontaneous Ep in unmanipulated blood from normal subjects and the relative insensitivity of the method used, the finding of Ep in over one third of SS patients indicates a significant membrane injury of the sickle RBC and serves to validate the in vitro observations. The possible role of the “senescence” mechanism in the induction of Ep

ISSN:0361-8609    

DOI:10.1002/ajh.2830200406

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractRecent evidence suggests that asialotransferrin may enhance hepatic uptake of trivalent metal ions through the asialoglycoprotein‐receptor pathway. In hereditary hemochromatosis, there is preferential uptake of iron by hepatocytes. We purified transferrin from two patients with this disease. Their transferrins had normal electrophoretic patterns and contained normal amounts of sialic acid. We conclude that the preferential uptake of iron by hepatocytes in hereditary hemochromatosis is not due to the presence of asialotransferrin in their plasm

ISSN:0361-8609    

DOI:10.1002/ajh.2830200407

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractWe have presented a retrospective study of 129 patients with platelet counts of 1,000 × 109/L or more—72 with myeloproliferative disorders (MPD), and 57 with reactive thrombocytosis (RT)—in an effort to determine the incidence of thrombohemorrhagic complications. Thrombotic disorders occurred with approximately equal frequency in the two groups, being found in three patients (4%) with MPD and three (5%) with RT when the platelet count was over 1,000 × 109/L and in 11% of the patients in each group when the platelet count was less than 1,000 × 109/L. In contrast, bleeding manifestations were more common in MPD, where 36% of patients were affected on one or more occasions versus only 4% of those with RT. However, bleeding was generally not severe, with only six patients requiring transfusions, and three of these patients had been receiving aspirin or corticosteroids when they developed severe gastrointestinal bl

ISSN:0361-8609    

DOI:10.1002/ajh.2830200408

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractHuman erythrocytes suspended in hypertonic solutions undergo hemolysis when the temperature of the suspension is changed from 37°C toward 0–4°C. It has been suggested that the hypertonic environment causes some proteins of the skeletal network to be changed in such a way that their normal adaptation to temperature changes is prevented, thus resulting in cryohemolysis. In the present study, we compared the cryohemolysis of some pathologic red blood cells in hypertonic sucrose and NaCl to normal cells. Erythrocytes of hereditary spherocytosis (HS) were found to be significantly more fragile than all others in hypertonic sucrose, while they behaved normally in hypertonic NaCl. In contrast, erythrocytes of thalassemic patients showed decreased susceptibility to cryohemolysis, both in hypertonic sucrose and in NaCl. Autoimmune hemolytic anemia samples behaved like normal samples, both in NaCl and in sucrose. The erythrocytes of congenital dyserythropoietic anemia‐type II patients showed two types of cryohemolysis; one pattern was similar to that of HS, and the other one presented normal levels in sucrose and reduced levels in NaCl. The different patterns of cryohemolysis described for the pathologic cells are thought to reflect different lesions in the membranes of the erythrocytes of the various hemolytic disorders. It is hoped that studying the cryohemolysis of abnormal red cells may contribute some illumination as to molecular interactions in intact cells in health and in di

ISSN:0361-8609    

DOI:10.1002/ajh.2830200409

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractWe investigated the optimal conditions for measuring glucocorticoid receptor in blast cells from patients with acute nonlymphocytic leukemia.Cytosol receptor measured with isoelectric focusing was saturated after 60 min of incubation at 0°C with 100 nM of either dexamethasone or triamcinolone. Saturation was achieved when cytosol from at least 7 × 106cells was used for incubation. Trypsin treatment of the cytosol resulted in a sharpened peak of receptor focusing at pH 5.6 with no loss of receptor‐bound radioactivity.The two physical forms of glucocorticoid receptor were isolated with DEAE cellulose chromatography. They were both found to focus at pH 5.6 during isoelectrc focus

ISSN:0361-8609    

DOI:10.1002/ajh.2830200410

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractWe present the clinical, pathologic, and metabolic findings of an adult woman with debilitating coronary artery disease and hepatosplenomegaly who was discovered to have multiorgan infiltration by sea blue histiocytes. A diagnosis of sea blue histiocyte (SBH) syndrome was made and no further workup performed. The patient suffered from progressive heart failure and sepsis following coronary artery bypass surgery and died 9 months after presentation. Tissues examined at autopsy showed pronounced infiltrates of both granular sea blue histiocytes and foamy, vacuolated histiocytes, which were morphologically compatible with Niemann‐Pick cells. Ultra‐structural examination of these cells revealed lamellar myelin‐like figures as described in Niemann‐Pick (N‐P) disease. Fibroblast enzyme assay studies and liver lipid analyses performed after the patient's death revealed pronounced sphingomyelinase deficiency and a lipid profile diagnostic of N‐P disease, type B. This case adds further support to the claim that some cases of apparent SBH syndrome actually represent a type of

ISSN:0361-8609    

DOI:10.1002/ajh.2830200411

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY