摘要:

Platelet function profiles were studied in 3 patients with megakaryoblastic leukemia. All patients had a moderate decrease in platelet counts with abnormal platelet retention. One patient who developed hemorrhagic diathesis had prolonged bleeding time. In all patients platelet aggregation was defective after the addition of ADP, collagen, adrenaline, or U46619, athromboxane A2 agonist. Malondialdehyde was reduced in all patients, as was platelet serotonin. Plasma β-thromboglobulin levels were normal in all cases whereas PF4 was markedly elevated in one. Platelet dysfunction was not reversed by clinical remission. These studies confirm that megakaryoblastic leukemia is associated with a thrombocytopathy which may play a role in hemorrhagic diathesis and should be taken into account in the management of these patients

ISSN:0001-5792    

DOI:10.1159/000205389

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

The leukemic promyelocytes in 37 cases of acute promyelocytic leukemia (APML; FAB, M3) were examined for their cytochemical property. Thirty-two cases (86%) showed strong myeloperoxidase (MPO), chloroacetate esterase (Es-chl) and Sudan black B (SBB) positivity, suggesting a pure neutrophilic differentiation of the leukemic cells. However, in 5 out of 37 cases, a strong, diffuse alpha-naphthyl acetate esterase (Es-a) positivity, which was sensitive to sodium fluoride treatment was observed in addition to strong MPO, Es-chl and SBB positivity. This suggested monocytic differentiation of a proportion of APML cases. In 31 cases, surface marker studies were carried out with the help of a panel of monoclonal antibodies consisting of two pan-myeloid antibodies (GM 58/8 and 1G10), one anti-HLA-DR antibody (7.2) and one ‘myeloid’ antibody (5F1) with restricted reactivity with monocytes (CD14). The purpose of including the monoclonal antibodies 5F1 and 7.2 was to determine if a correlation could be established between strong Es-a positivity and reactivity of the leukemic promyelocytes with 5F1 and 7.2 in individual cases. All the 5 cases with ‘monocytoid’ cytochemistry were unreactive with 5F1, and only one case in this group showed 15% 7.2-positive cells. The lack of immunophenotypic support for the monocytic cytochemistry of the 5 cases of APML suggests that the monocytic phenotype of leukemic promyelocytes is both aberrant and incomplete. Since normal promyelocytes are purely neutrophilic, this could be a manifestation of an ‘intralineage infidelity’ in APML, similar to that observed in other types of acute leukemia. The clinical significance of ‘monocytic’ phenotype of APML is not clear; cases with monocytic differentiation did not show different clinical and hematological features when compared to the more common, pure neutr

ISSN:0001-5792    

DOI:10.1159/000205390

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

A new in vitro colony growth assay system method was found to be reliable in its use for evaluating B-cell proliferation in normal subjects and in 9 patients with B-cell chronic lymphocytic leukemia (B-CLL). The method is based on a phytohemagglutinin (PHA)-stimulated monocyte and T-cell-conditioned medium (PHA-MTCM) composed of PHA, silica, normal monocytes and normal T cells. The number of colonies proliferated was significantly greater in 5 patients who had not undergone treatment than in normal subjects (1,417 ± 660 vs. 661 ± 119) (p < 0.002). Normal cultured B-cell colonies were shown to be 71% surface IgM colonies, and 6% cytoplasmic IgA colonies with the appearance of blastic cells. B-CLL colonies, on the other hand, were demonstrated to be monoclonal with the same CLL circulating cells being retained. We also studied the effect of interleukin-2 (IL-2) on B-cell colony growth assay in 4 patients with B-CLL cells. Only 1 patient with M protein responded to IL-2, proliferated and expressed IL-2 receptors. Although 3 patients without M protein did not respond to IL-2, they did respond to the supernatant, and they proliferated but expressed no IL-2 receptor

ISSN:0001-5792    

DOI:10.1159/000205391

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Plasma samples from patients with disseminated intravascular coagulation (DIC) associated with acute promyelocytic leukemia (APL) exhibited higher levels of the D-fragment of fibrin and fibrinogen degradation products [FDP(D)], with relatively lower levels of cross-linked fibrin degradation products (XDP), than samples of DIC with non-APL. The difference between FDP(D) and XDP levels increased only when α2-plasmin inhibitor (α2-PI) fell below 60% of the normal level in APL patients. These findings suggest that fibrinogenolysis occurs in APL patients when the α2-PI level has decreased significant

ISSN:0001-5792    

DOI:10.1159/000205392

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

In order to assess whether changes in the relative proportions of cells pertaining to different T-lymphocyte subsets correlate with dipeptidyl amino peptidase IV (DAP IV) activity, the enzyme was cytochemically investigated in circulating lymphocytes from 20 HIV-I-seropositive drug addicts. Although on the average, a significant reduction of lymphocyte DAP IV activity was found in comparison with healthy control subjects, wide variations between individual cases were observed, and no obvious relationship emerged between DAP IV staining on one hand, immunocytological findings and Walter Reed staging on the other. Therefore, in view of the preliminary results reported, the variations of lymphocyte DAP IV activity in the course of HIV I infection seem to deserve further investigation, in order to clarify both the biological significance of this reaction and its possible clinical relevance.

ISSN:0001-5792    

DOI:10.1159/000205393

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

A total of 38 patients with β-thalassemia intermedia from 30 families were studied. Twelve of the thirty unrelated patients had β°-thalassemia which was due to a homozygosity for one of two different thalassemia defects, namely the frameshift at codon 8, and the IVS-II-1 G→ A mutation. Another mild variation, a β +-thalassemia, was a homozygosity for the mutation of T→C at position 6 of IVS-1 (10 patients). Compound heterozygosities for mild thalassemic determinants or for one mild and one severe β-thalassemic determinant were also found in some patients with β-thalassemia intermedia. The mutations at β-39 and IVS-I-110 were the most commonly occurring thalassemic determinants in these patients. Correlations between genotype and phenotype indicated significant differences in some of the hematological parameters among patients with the IVS-I-6 and the frameshift at codon 8, IVS-I-6 and IVS-II-1, and the frameshift at codon 8 and IVS-II-1

ISSN:0001-5792    

DOI:10.1159/000205394

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Sixteen patients with chronic idiopathic thrombocytopenic purpura underwent splenectomy after failure of steroid therapy. The average time of follow-up was 69 months. Immediately after splenectomy, complete response (platelets above 150 × 109/1) was obtained by 68% of patients while 25% had partial response (platelets from 50 to 150 × 109/1) and only in 1 patient splenectomy failed. During the long-term follow-up, 2 patients relapsed 9 and 20 years, respectively, after splenectomy (20% of non-responders). Partial recurrence of thrombocytopenia (partial response) was observed in 33% of the patients. The persistent complete response rate was then 47%. The young patient age appears to be the only positive predictive factor both for short-term and long-term response to splenectomy. The platelet recovery rate and postsplenectomy thrombocytosis are also early features of lasting response. Positive serum and platelet-associated immunoglobulins became negative after splenectomy, but there is no correlation with clinical respons

ISSN:0001-5792    

DOI:10.1159/000205407

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Two subsequent series of patients with Hodgkin’s disease (HD) treated according to different therapeutic plans were compared: the study made it possible to analyze the role played by therapy in influencing the individual importance of a group of well-known prognostic factors. Study 1 concerned 667 patients treated in the period 1971–1979 without special measures for mediastinal bulky disease and with four-drug chemotherapy regimens (MOPP, COPP, ABVD) for stage B or IV. Study 2 included 220 patients treated between 1980 and 1984 with combined sandwich chemoradiotherapy when mediastinal bulk was present, and with eight-drug alternating chemotherapy regimens for stages B or IV (MOPP/ ABVD, CcVPP/ABVD). Distribution of epidemiologic and clinical characteristics as well as staging accuracy were comparable in the two series. Only sex, serum albumin at onset and success or failure in achieving complete remission showed the same ability to discriminate survival in both studies. Age, stage and histology retained a reduced role in Study 2, where it was found they could be handled as binary variables, i.e. more or less than 50 years of age, stage IV or other stages, lymphocyte depletion histotype or other types. The influence of B symptoms on survival was sharply decreased in patients treated with alternating chemotherapy regimens, whereas combined sandwich therapy showed a truly leveling effect on the role of mediastinal bulk, which has to be considered a very unfavorable factor with other treatments. In HD the evaluation of clinical findings with respect to their impact on prognosis is crucial for validating and graduating the staging process, and for matching the intensity of the therapy to the needs of the patient. The ongoing evolution in the roles of single prognostic factors due to therapy needs periodic reevaluation for proper adjustments of therapeutic strateg

ISSN:0001-5792    

DOI:10.1159/000205397

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

In this report we describe a case of granulocytic sarcoma (GS) developing in a patient after 66 months in complete remission of acute nonlymphoid leukemia. The granulocytic precursor cell proliferation arose simultaneously in two extramedullary sites (testis and small bowel), without evidence of bone marrow relapse. The intensive systemic chemotherapy with high-dose ARA-C allowed a clinical remission, lasting 8 months. GS eventually recurred as extramedullary multi-site disease (abdominal lymph nodes, central nervous system involvement), once again unassociated with blood and bone marrow relapse. Both, at onset and in relapse, special staining techniques on tissue sections (chloroacetate esterase) and immunohistochemical typing with monoclonal antibodies were necessary for a correct diagnosis. We discuss the relationship between systemic leukemia and the GS which showed a metastasizing tumor-like behavior.

ISSN:0001-5792    

DOI:10.1159/000205398

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Primary malignant lymphoma of the central nervous system (CNS) is rare and only infrequently involves the spinal cord, usually in association with multifocal CNS spread. We report a case of primary lymphoma infiltrating lumbar nerve roots without evidence of systemic lymphoma. There has been an excellent response to combined modality therapy, comprising radiotherapy with intrathecal and intravenous cytotoxics. The patient remains well and active 22 months after diagnosis. The literature regarding these rare tumors is reviewed.

ISSN:0001-5792    

DOI:10.1159/000205399

出版商:S. Karger AG    

年代:1989

数据来源: Karger