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A diagnosis of acute lymphoblastic or myeloblastic leukemia was made in 6 children ages 4 to 14 years who presented with a clinicobiochemical picture of acute hepatitis without liver failure. Standard chemotherapy, including 1 week pretreatment with steroids in children with lymphoblastic leukemia, allowed complete remission of the leukemia and normalization of serum liver tests.

Transformation of myelodysplastic syndrome to hematologic malignancies is not uncommon, but involvement of three committed progenitor cell lines at one time is seldom seen. We present a case of myelodysplastic syndrome with blast transformation, neoplastic cells that were difficult to classify morphologically, and basophilia found in marrow and peripheral blood. Differential diagnosis included myeloblastic leukemia, erythroblastic leukemia, megakaryoblastic leukemia, basophiloblastic leukemia, and systemic mast cell disease (SMCD). By using a battery of cytochemical techniques niques and irnrnuno~tochemicalm ethods, we were able to demonstrate the presence of myeloblasts, erythroblasts, and megakaryoblasts, thus excluding the possibility of SMCD and basophiloblastic leukemia. (The J Histotechnol 13:67, 1990)

A case of acute undifferentiated myelocytic leukemic with trisomy 4 is described. The patient is a 61-year-old woman who developed leukemia 4 1 2 years after receiving radiation therapy for uterine carcinoma. Many leukemic cells exhibited hand-mirror configuration after the bone marrow aspirate was left at room temperature overnight. The relationship between trisomy 4 and hand-mirror cells in acute myelocytic leukemia is unknown.

The unusual presentation of acute myeloblastic leukemia as a scapular granulocytic sarcoma in an infant without systemic manifestations is reported for the first time. Granulocytic sarcoma as a presentation of leukemia should be considered in the differential diagnosis of scapular masses during childhood. Surgery is limited to obtain sufficient tissue for histopathologic diagnosis.

In this retrospective study of 62 children with ALL and 10 children with AML, data are compiled on the special aspects of a guarded indication for platelet transfusions; in Tubingen this indication is usually based solely on clinical signs of incipient or manifest bleeding at platelet counts below 11,000/mm3. This is in contrast to the practice most often used to transfuse platelets at counts below 20,000/mm3 for prophylactic reasons irrespective of therapeutic need. The guarded indication aims at keeping the rate of allosensitization by platelet transfusions as low as possible. Of all patients in remission induction of ALL and of AML, platelet transfusions were needed by 53% and 80% respectively; the mean platelet support was 2.9 units in ALL and 19.0 units in AML. In relapse, the need increased considerably. Of all patients with ALL, 11% were sensitized by platelet transfusions; lethal complications related to this guarded indication could not be seen.

Decreased nuclear segmentation and coarse chromatin only in mature eosinophils was seen in a patient with acute myeloblastic leukemia. These eosinophils were considered to have acquired Pelger-Huet anomaly. On cytochemical study they displayed a strong PAS-positive reaction.

The relation between the quantified French-American-British cooperative study (FAB) classification and presenting features was studied in 123 adult patients (greater than or equal to 15 years) with acute myeloid leukemia. Myeloblastic leukemia with maturation (M2) patients with a high maturation index (M2a) were older, compared to the other myeloblastic subgroups, while promyelocytic leukemia (M3) patients were younger than those with M2 or M2a. Differences between various leukemia types were found in the occurrence of a preceding myelodysplastic syndrome, leukocyte count, and platelet count. In both monoblastic leukemia (M5) and myeloblastic leukemia (M1-3) a smaller degree of maturation seemed to be related to a larger tumor mass. The effect of chemotherapy was analyzed in a group of 64 patients. Comparison between immature (M1) and more mature (M2) myeloblastic leukemia suggested a longer duration of first complete remission and a longer survival in M2. The findings of this study may imply that the degree of maturation determines tumor mass and the success of chemotherapy.

We refer to 48 adult patients suffering from acute myeloblastic leukemia, whose leukemic cells showed a typical cytochemical pattern: i.e. weak staining for peroxydase and with sudanblack B and lacking or only weak staining for nonspecific esterase. In 8 patients the leukemic myeloblasts additionally showed distinct granular staining for polysaccharides using the PAS-reaction. The therapeutic response, the remission rate and the survival time of these 8 cases have been compared to those of 40 patients, whose leukemic myeloblasts differed exclusively in the absence of granular PAS-positive materials. Out of the latter PAS-negative cases 7 patients (18%) went into a complete remission (M1, P1), 5 patients achieved partial remission (M2, P1-2), the 50%-survival time was 4.9 months. Out of the 8 granular PAS-positive cases 4 patients (50%) went into complete (M1, P1), and 1 reached partial remission (M2, P1-2). The 50%-survival time of the cases now lasts 10.1 months and three patients are still alive and in persisting complete remission 19, 12.5 and 12 months after diagnosis. These results suggest a better prognosis and an improved therapeutic response in those myeloblastic leukemias which additionally contain cytoplasmic granular PAS-positive materials.

Three-hundred and ninety-nine patients with acute leukemia examined during the period 1947-1964 were divided into three sequential series and their survival, from diagnosis to death, was compared. A statistically significant increase in duration of survival for myeloblastic leukemia from a median of 2 to 5 months occurred between series I and series III. This is the first convincing evidence that 6-mercaptopurine therapy influences survival in myeloblastic leukemia. A steady increase in median survival of patients with lymphoblastic leukemia from 4 to 8 to 12 months was found in the three series. The results of analysis of these series are compared to other reported series. The hypothesis —that the longer survivals reported for patients treated in hematology clinics compared to that of patients selected from population areas reflects patient selection—was examined and appeared unlikely.

The longevity, reckoned from the symptomatic onset of acute leukemia as well as from the initiation of treatment, of a group of 89 cases including children and adults, treated in this clinic since March, 1954, was found to exceed that of a similar group of 78 patients treated in preceding years in the same clinic. The median survival of all cases considered as a unit was increased from three to six months, as measured from the date treatment was begun. The change was found to be statistically significant as well as clinically important. The longer survival found in the 1954-57 series correlated with the greater use of three, rather than only two, chemotherapeutic agents. On analysis, improved survival was statistically proven only for individuals with lymphoblastic leukemia; for these, the median survival was 10 months after the symptomatic onset of leukemia and 7½ months after the start of treatment. Within this group, there was an even greater improvement for those whose leukocyte values immediately prior to the initiation of therapy were less than 10,000 per cu.mm.; half of these patients lived more than 12 months after treatment was begun. The reason for the exceptional longevity of patients with the lower pre-treatment leukocyte values is unknown. It appears that one can attribute at least part of the improvement to therapy. The type of statistical analysis used presents the minimum figures for improvement in median survival. When the ultimate longevity is known for the 17 per cent of the cases surviving at the closing date of the study an additional small increment may be found. The relative inefficacy of chemotherapy in myeloblastic leukemia should be a signal for greater screening of new agents in this type of tumor.

The apparent protein content of a single spherical lymphocyte or a similar cell, as well as its diameter, can be measured by a modified and stabilized AO-Baker interference microscope which is either fitted with an AO half-shade eyepiece or connected to a photomultiplier. This paper gives results for the apparent protein content and the diameter of normal mouse lymphocytes and thymocytes; these do not differ significantly from each other. It also gives values for the apparent protein concentration and the diameter of the lymphocytes of mice of the same strain which have developed leukemia or lymphoma; these values are significantly different from those of normal mice. Related data are given for the apparent protein content and for the diameter of normal human lymphocytes and the white cells found in myeloblastic leukemia and in stem cell leukemia in man; here the values differ significantly from those of the normal human lymphocyte. The rule seems to be that the abnormal white cells become more watery and larger as the disease advances.

A case with a prolonged period of blood disorder preceding the terminal development of myeloblastic leukemia is reported in some detail. The preleukemic period was characterized by anemia, neutropenia and thrombocytopenia. It extended over a period of almost nine years but was punctuated by a period of remission lasting four and one half years. In the initial phase, the marrow was hypoplastic but during the later stages, it was hyperplastic. Brief reference is made to somewhat similar cases that have been recorded and to the ideas expressed by the authors of these reports. It is suggested that further investigation is needed to determine whether the initial blood disease occurring in such cases is irrevocably linked to the subsequent leukemia.

A case of a patient is presented, who suffered from acute leukemia, showing predominantly atypical myeloblasts in the bone marrow. In contrast, a high amount of atypical “neutrophils” were present in the peripheral blood, as well as there was a conspicuous number of monocyte-like intermediate forms between the myeloblasts and the atypical segmented “neutrophils.” The cytochemical pattern of the “Neutrophils” was highly atypical, the main part being devoid of sudanblack B-positive lipids, as well as of peroxidase and chloroacylesterase activity, which characteristically are present in large amounts in the neutrophil granulocytes. Moreover, atypical distribution of alkaline phosphatase and naphthylamidase activities were encountered in these cells. The intermediate or transitional cells exhibited a cytochemical pattern different from that of normal blood monocytes, but corresponding to that of the atypical neutrophils. Thus, the assumption of a direct maturation way from myeloblasts to “paraneutrophils” with mononuclear, monocyte-like intermediate stages was supported by the results of cytochemical investigations. In contrast to the elevated number of neutrophils in the peripheral blood (10 350/mm3) very rare normal neutrophil precursors with typical cytochemical pattern were encountered in the haematopoietic organs.

Four cases of severe refractory anemia with an enormous increase of demonstrable free iron in the normoblasts are reported. Three cases were of unknown etiology and of benign course but in the fourth the anemia was probably related to rheumatoid arthritis or to the onset of myeloblastic leukemia. The significance of the sideroblastic bone marrow in these cases is discussed and the opinion expressed in the literature—that it indicates a defect in hemoglobin synthesis is subscribed to. Thus, it is believed that the anemia in these cases is due to degenerative changes in the bone marrow which also have some morphologic manifestations. The disturbance of marrow activity is probably best reflected in the reticulocyte counts.

Summary Rubidomycin was administered in doses of 1 mg/kg body weight, usually in combination with prednisone. The best results were obtained in promyelocytic leukemia. Promyelocy-tic leukemia in children seems to be characterized by sensitivity to rubidomycin even in late exacerbations.

A detailed study (phase contrast, bright field and electron microscopy) of the primitive leukemic cells from a patient with atypical myeloblastic leukemia has been presented. An unusual mucopolysaccharide-containing vacuole has been observed and characterized both morphologically and histochemically. In addition, this study has yielded considerable information concerning the structure and chemical composition of the fibrillar formations, fat droplets and the alterations in erythrocytes following their ingestion by the leukemic cells. Intramitochondrial structural alterations have been observed and characterized in many of the primitive myeloblasts. Changes in the mitochondria and endoplasmic reticulum may reflect impaired or altered metabolism in these abnormal leukemic cells.

A patient with coexistent Gaucher disease and Philadelphia positive chronic granulocytic leukemia (CGL), who subsequently developed myeloblastic leukemia, is described. The diagnosis of CGL was established according to standard clinical, morphological, biochemical, and cytogenetic data, while the diagnosis of true Gaucher disease was based on biochemical data and the presence of Gaucher cells with typical ultrastructural features in the bone marrow and spleen. Enzyme studies showed low activity of ceramide-β-glucosidase in the patient's peripheral blood leukocytes, skin fibroblasts, and splenic tissue and the presence of increased amounts of ceramide-β-glucoside in the spleen. This case is reported in order to draw attention to the possible coexistence of these two diseases in the same patient, as opposed to the well-recognized finding of “Gaucher-like” cells in the bone marrow of patients with CGL. Enzyme studies enable distinction between these two situations.

A PATHOGENIC relation between aplastic anemia and myeloblastic leukemia has long been suspected. It is well known that certain agents capable of producing aplastic anemia in man can also induce leukemia. Prime examples are benzene1 , 2 and irradiation.3 , 4 In addition, cases of leukemia developing during the course of hereditary hypoplastic syndromes have been recorded.5 , 6 The broad-spectrum antibiotic chloramphenicol7 8 9 has become the most commonly cited cause of acquired aplastic anemia. To date, only 1 case report of acute myeloblastic leukemia following aplasia secondary to chloramphenicol administration has been published.10 The purpose of this communication is to present 2 additional cases of chloramphenicol-induced . . .

SURVIVAL in myeloblastic leukemia of adults has not appreciably changed since the advent of chemotherapy. As pointed out by Gunz et al.,1 the progress of the disease may be quite variable, but it usually follows a rapidly downhill course, with death ensuing within several weeks to months. A few patients, however, may live in apparent symbiosis with their disease for periods of up to several years. These cases, unfortunately, are uncommon. Recently, several reports have appeared suggesting that aggressive chemotherapy with combinations of agents may prolong life in this disease.2 3 4 To evaluate these studies in the light of our own . . .

Excerpt To the editor: In his recent editorial, Gale (1) kindly mentioned the randomized trial on myeloblastic leukemia (consolidation chemotherapy versus no consolidation) of the Spanish Cooperati...