American Journal of Hematology

doi: 10.1002/ajh.2830080401pmid: N/A

Kutti, Jack; Zaroulis, Charles G.; Kulpa, Jolanta; Reich, Lilian; Clarkson, Bayard D.; Good, Robert A.

doi: 10.1002/ajh.2830080402pmid: 6158265

Plasma beta‐thromboglobulin (β‐TG) levels were measured in 14 healthy subjects and in 20 acute leukemia (AL) patients, newly diagnosed, with highly variable values for venous platelet counts. For healthy subjects the plasma β‐TG levels ranged 12–38 (mean 17) ng/ml. In this group of patients with AL, a highly significant positive correlation (P < 0.001) between the values for plasma β‐TG and venous platelet count was present. During a thrombocytopenic period, the plasma β‐TG concentration was measured in nine of the AL patients immediately before and 10 to 12 hours after platelet transfusion therapy. Fourteen platelet transfusions were administered when the patient's highest temperature of the day was <38.5° C, and 18 when the highest temperature of the day was <38.5°C. The mean pretransfusion and post‐transfusion β‐TG values for the 14 platelet transfusions were 7 ± 2 and 20 ± 5 ng/ml, respectively. The corresponding means for the 18 transfusions given to febrile patients were 5 ± 2 ng/ml and 11 ± 2 ng/ml, respectively. Of the pretransfusion values, 11/14 and 14/18 were below the control range. We conclude that the plasma β‐TG values are considerably lower in thrombocytopenic patients than in subjects with normal platelet counts. Further work should provide reference values for plasma β‐TG over a wide range of venous platelet counts.

Daly, Peter A.; Schiffer, Charles A.; Wiernik, Peter H.

doi: 10.1002/ajh.2830080403pmid: 6932177

Fifteen patients with acute promyelocytic leukemia (APL) were treated with anthracycline agents alone or combined with arabinosylcytosine (Ara C). Disseminated intravascular coagulation (DIC) was managed individually based on the presence of clinical as well as laboratory evidence of DIC. Ten patients received heparin. Eleven patients (73%) achieved complete remission (CR) but there were two early deaths from infection. The median duration of CR was 232 days (range, 41–780 days). Six patients were studied during ten relapses. In all cases, a morphologic picture more typical of acute myelocytic leukemia was seen during relapse but the DIC was similar in severity to that seen initially. Fever, without obvious infection and possibly due to the leukemic process, was a common presenting feature. Regenerating bone marrow aspirates in many patients who subsequently achieved CR without further treatment showed an inordinate number of promyelocytes, and this finding should not be immediately interpreted as disease resistant to therapy.

Potter, Jane E. R.; Wright, Eric G.

doi: 10.1002/ajh.2830080404pmid: 7416163

The lipids in the bone marrow of six strains of normal mice, and mice with genetically determined anemias (W/Wv and S1/S1d) and experimentally induced anemia were analyzed. Cholesterol and phospholipid accounted for 60–75% of total lipid; the remainder was triglyceride and neutral glyceryl ether lipids. In mice with phenylhydrazine (PHZ)‐induced hemolytic anemia there was a threefold increase in neutral glyceryl ether lipids immediately preceding a twofold increase in recognizable erythroid precursors. Comparisons were made between the femoral bone marrow and the cells maintained in the adherent populations of long‐term bone marrow cultures. In cultures of B6D2F1 bone marrow (which support long‐term proliferation and differentiation of hemopoietic stem cells) the adherent layers contained the same lipids as freshly aspirated bone marrow. In W/Wv and S1/S1d cultures (which duplicate, respectively, the stem cell and environmental defect found in vivo) the lipid content of the adherent layers differed from bone marrow, particularly in the absence of ether‐containing lipids. The results are consistent with the hypothesis that marrow lipids have an active (as opposed to passive) role in murine hemopoiesis and indicate that long‐term bone marrow culture may prove useful in further biochemical studies of the hemopoietic inductive microenvironment.

Narasimhan, Parthasarathy; Amaral, Leonard

doi: 10.1002/ajh.2830080405pmid: 7416164

Three patients with chronic lymphocytic leukemia (CLL) and carcinoma of the prostate were treated for the latter condition with diethylstilbestrol (DES). All three patients responded with rapid reductions in their peripheral white counts. The previously observed lymphocytic infiltration of the bone marrow of one patient was not evident after three months of DES treatment. The response of this patient to DES correlates well with the enhanced level of plasma transcortin subsequent to treatment. Treatment of this patient with DES alters the ability of peripheral lymphocytes to incorporate radioactive uridine into RNA as well as the in vitro response to either cortisol or β‐estradiol.

Litosch, Irene; Lee, Kwang Soo

doi: 10.1002/ajh.2830080406pmid: 6448000

Sickle (Hb SS) red cells, preloaded with 45Ca by reversal of hemolysis, exhibit an incomplete 45Ca extrusion, retaining approximately four times more 45Ca than normal cells. Studies indicated that neither the reduction in Hb SS cell Ca2+‐Mg2+ ATPase activity (84% of normal) nor the activation of Ca2+‐Mg2+ ATPase by calmodulin was sufficiently different from normal cells to attribute a major role to the calcium pump in 45Ca retention. These results suggested that 45Ca retention may reflect an alteration in the calcium‐binding properties of Hb SS cell membranes. Low‐affinity calcium‐ binding (freely dissociable) was similar in normal and Hb SS cell membranes. However, the total calcium bound with high‐affinity (tightly bound) was four‐to‐five times greater in Hb SS cell membranes than in normal membranes. These results are compatible with the hypothesis that Hb SS cell 45 Ca retention reflects an exchange of a fraction of the total 45Ca with a tightly bound calcium pool, larger in Hb SS cell membranes than in normal membranes. A comparable degree of red cell 45Ca retention, which did not correlate with the reticulocyte population, was observed in other chronic anemic states. These findings suggest that the increased high‐affinity calcium binding by the membrane may be a consequence of cellular changes induced by the anemic condition.

Lubiniecki, A. S.; Blattner, W. A.; Dosik, H.; McIntosh, S.; Wertelecki, W.

doi: 10.1002/ajh.2830080407pmid: 6251720

Skin fibroblasts from patients with a variety of hematologic disorders were infected with SV40 virus in vitro in attempts to discover the reason for increased susceptibility of Fanconi anemia cells to this transforming virus. The proportion of skin fibroblasts expressing SV40 T‐antigen by immunofluorescent methods was elevated in 12 patients with Fanconi anemia and in seven of nine obligate heterozygous relatives. Elevated expression was also observed in three patients with other hematological disorders at high risk of acute nonlymphocytic leukemia, but was not apparent in seven sporadic aplastic anemia patients or four of their relatives. T‐antigen expression was elevated in about one‐half of patients with thrombocytopenia‐absent radius syndrome and related conditions, with familial aplastic anemia, and in their normal relatives. In the conditions under study, elevated T‐antigen expression seemed clearly correlated with predisposition to leukemia, which may be genetically determined, but it was not associated with cytogenetic or anemic manifestations.

Ness, P. M.; Hymas, P. G.; Gesme, D.; Perkins, H. A.

doi: 10.1002/ajh.2830080408pmid: 7416165

Two cases of Hansen disease demonstrating a lupus‐like inhibitor directed against the activation of coagulation factor X are described. Each case showed factor‐X activity markedly depressed by an inhibitor when measured in one assay system; yet normal levels were measured by three alternate factor‐X assay systems.

Morosawa, Hironori; Yamazaki, Munehiro; Sugita, Kenichi; Saitoh, Hiroharu; Komiyama, Atsushi; Akabane, Taro

doi: 10.1002/ajh.2830080409pmid: 6968158

A boy with recurrent pyogenic infection was found to have occasional neutropenia, defective neutrophil chemotaxis, hypogammaglobulinemia with increased IgM, and impaired cellular immunity. The T and B lymphocytes were defective in IgG production in vitro. Ultrastructure of the neutrophils was normal. The marrow cells formed normal numbers of granulocytic colonies in culture, but the colonies were apparently small in size. The levels of colony‐stimulating activity were normal. The lymphocytes did not impair granulopoiesis of control marrow cells. These data indicate that the neutropenia and defective neutrophil chemotaxis are due to the intrinsic neutrophil defects and are not secondary to T and/or B lymphocyte dysfunctions in the patient.

Moore, Charleen M.; Weinger, Ronald S.

doi: 10.1002/ajh.2830080410pmid: 7416166

An individual with normal male habitus, body proportions, and secondary sexual characteristics was admitted to the hospital with head trauma. A routine blood smear demonstrated that 36% of the granulocytes had “drumsticks.” Chromosomal analysis revealed a 46, XYqh+ karyotype. The extremely large Y chromosome was located by quinacrine fluorescence in the “drumstick” of the polymorphonuclear granulocytes. The presence of a large Y chromosome may thus produce pseudo‐drumsticks. Fluorescent staining can distinguish between true drumsticks bearing the inactive X of normal females and the pseudo‐drumsticks in a normal male produced by a large Y chromosome.