Clinical usefulness of immunocytochemical detection of myeloperoxidase in undifferentiated and biphenotypic acute leukemias.

BACKGROUND. The use of monoclonal antibodies has been recently proposed as a more sensitive technique than conventional cytochemistry in recognizing myeloperoxidase (MPO) in leukemic cells. METHODS. In order to verify the diagnostic relevance of such an approach, the immunocytochemical detection of MPO by means of the APAAP technique and a monoclonal antibody directed against an antigenic determinant of MPO (MPO7) was performed in 10 patients with undifferentiated or biphenotypic (myeloid and lymphoid) acute leukemia. All the cases were negative for conventional cytochemical staining of MPO. RESULTS. Positive cells (15 to 65%) were found in three patients. Some clinical data and biomolecular studies of T-cell receptor and immunoglobulin genes, available in five patients, indirectly confirmed these findings. CONCLUSIONS. Although some technical and biological aspects remain to be better characterized, immunocytochemical methods which utilize monoclonal antibodies against MPO may represent a simple and reliable tool for improving the diagnostic capacity to identify undifferentiated myeloid blasts in difficult cases of acute leukemia.     

MAE方案联合化疗治疗CD7^＋急性髓性白血病疗效观察

目的：探讨应用米托蒽醌 阿糖胞苷 足叶乙甙（MAE）方案治疗CD7^ 急性髓性白血病（CD7^ MAL）的有效性。方法：采用MAE方案联合化疗治疗经细胞形态学分型诊断为CD7^ AML12例,并监测化疗后骨髓像变化及化疗相关毒性反应。结果：12例中,7例达完全缓解,1例达部分缓解,总有效率为66.7%,持续缓解时间6-36个月。化疗相关毒性各大于HA和DA方案,主要特征为骨髓抑制期延长。结论：MAE方案可提高CD7^ AML的缓解率,尽管化疗后骨髓抑制期延长,但能被患者耐受。     

Biological characteristics of CD7 positive acute myelogenous leukaemia

We studied the biological characteristics of CD7+ acute myelogenous leukaemia (AML). We diagnosed nine out of 88 consecutive AML cases as CD7+ AML based on myeloperoxidase positivity and surface antigen expression. In eight of these nine cases more than 20% of leukaemic blasts were found to coexpress both CD7 and a myeloid-associated antigen, CD33, by a two-colour flow-cytometric assay, while in the remaining case more than 90% of blasts were positive for CD7 and myeloperoxidase. CD7+ AML was most frequently observed in M1 among AML subtypes according to the FAB classification. An early stage-specific antigen, CD34 was also expressed on leukaemic blasts from eight of these nine cases. Neither the T-cell receptor (TcR)-beta nor the TcR-gamma gene was clonally rearranged in any of the cases. We then studied the proliferative responses to stimulation by various growth factors. Among interleukin-3 (IL-3), granulocyte/macrophage colony-stimulating factor (GM-CSF), and granulocyte-CSF (G-CSF), IL-3 showed the strongest stimulatory effect on DNA synthesis and leukaemic blast colony formation in 8/9 and 6/8 CD7+ AML cases examined, respectively. On the other hand, the strongest stimulatory effect exerted by IL-3 on blast colony formation was observed in only six out of the 33 CD7- AML cases examined. Furthermore, CD7+ AML blasts could proliferate in response to stem cell factor (SCF); SCF alone showed stimulatory effects on blast colony formation (7/8 cases), and in 5/7 SCF-responding cases, stimulatory effects of SCF were more potent than those of IL-3. In addition, SCF enhanced blast colony formation synergistically with IL-3 in four of these seven cases. These data suggest that progenitor cells of CD7+ AML may possess the biological properties characteristic of immature haematopoietic stem cells.     

Clinical and biologic features of CD4(+)CD56(+) malignancies

CD4(+)CD56(+) malignancies are rare hematologic neoplasms, which were recently shown to correspond to the so-called type 2 dendritic cell (DC2) or plasmacytoid dendritic cells. This study presents the biologic and clinical features of a series of 23 such cases, selected on the minimal immunophenotypic criteria defining the DC2 leukemic counterpart, that is, coexpression of CD4 and CD56 in the absence of B, T, and myeloid lineage markers. Clinical presentation typically corresponded to cutaneous nodules associated with lymphadenopathy or spleen enlargement or both. Cytopenia was frequent. Circulating malignant cells were often detected. Massive bone marrow infiltration was seen in 20 of 23 (87%) patients. Most tumor cells exhibited nuclei with a lacy chromatin, a blastic aspect, large cytoplasm-containing vacuoles or microvacuoles beside the plasma membrane, and cytoplasmic expansions resembling pseudopodia. Other immunophenotypic characteristics included both negative (CD16, CD57, CD116, and CD117) and positive (CD36, CD38, CD45 at low levels, CD45RA, CD68, CD123, and HLA DR) markers. The prognosis was rapidly fatal in the absence of chemotherapy. Complete remission was obtained in 18 of 23 (78%) patients after polychemotherapy. Most patients had a relapse in less than 2 years, mainly in the bone marrow, skin, or central nervous system. Considering these clinical and biologic features, the conclusion is made that CD4(+)CD56(+) malignancies constitute a genuine homogeneous entity. Furthermore, some therapeutic options were clearly identified. Finally, relationships between the pure cutaneous indolent form of the disease and acute leukemia as well as with the lymphoid/myeloid origin of the CD4(+)CD56(+) malignant cell are discussed.     

Clinical and cytological features of acute myelogenous leukemia with 8; 21 chromosome translocation

Eight cases of acute myelogenous leukemia with (8; 21) translocation were reported. As recently reported, they showed following features: M2 morphology in FAB classification (all 8 patients), abnormal granulocyte maturation, i.e. large granules and pseudo Pelger-Huet forms (5), Auer rods (8), occasional eosinophilia (2), frequent loss of one sex chromosome (5), the low neutrophil alkaline phosphatase activity (5), and tumor formation (one). Both CD13 and CD33 antigens were expressed on smaller number of leukemic cells than the other AML (M2) cells, whereas CD34 and HLA-DR antigens were expressed on higher number of cells. Interestingly CD19 antigen was detected on a small to large population of tumor cells from four out of six patients. Despite the high remission rate, many of them relapsed within one year. More intensive postinduction and maintenance therapy should be considered for those patients.     

Clinical features of eosinophilic gastroenteritis]

BACKGROUND/AIMS: Eosinophilic gastroenteritis (EG) is an uncommon disease with various gastrointestinal symptoms characterized by eosinophilic infiltration. Its incidence increases recently. We investigated the clinicopathologic features of the patients with EG. METHODS: Between January 1970 and July 2003, a total of 28 cases of EG have been reported. By adding 3 cases diagnosed in the Eulji Hospital, we evaluated the 31 cases of EG for the symptoms, laboratory findings, histologic findings, method of treatment, and allergic history. RESULTS: The incidence increased during 1990s. The predominant site of the eosinophilic infiltration was mucosa in 10 cases, muscularis in 5 cases, and subserosa in 5 cases. The remaining 11 patients had the EG in two sites. The most frequently affected organ was small intestine (12 patients), but there were 12 cases which more than two organs involved. Melena and hematochezia were dominant findings in the patients whose infiltration occurred in mucosa. On the other hand, ascites was a dominant finding in the cases that infiltration occurred in subserosa or involving over 2 layers. There was no difference in eosinophilia, clinical symptoms, and duration between two groups according to the symptom duration for less or more than 30 days. Additionally, seven patients had allergic histories. Peripheral eosinophilia was found in 24 cases. There was no recurrence of EG after the treatment. CONCLUSIONS: Our retrospective studies about EG reported in Korea could not clarify whether EG is related to allergy. Recently, the occurrence of EG is increasing and it is frequently involved in young men. The most apparent finding include peripheral eosinophilia. There is no significant clinical feature which is consistent with Klein's classification, though blood loss was more common in mucosal type and ascites was more common in serosal type and in case above two layers.     

Clinical features of childhood Ph1 positive acute leukemia

Clinical, immunologic, cytogenetic and molecular studies were performed on 9 patients with childhood Ph1 positive acute leukemia. FAB-L1 was found in 2 patients, L2 in 5 patients, and M1 and M2 in each patient. Six patients were older than 10 years old, and white blood cell count of 5 patients was more than 10(5)/microliters. All but one patient have died within 18 months. Immunologic analysis revealed that leukemic cells from all patients expressed lymphoid antigens CD10 and CD19, and myeloid antigen, CD13, was expressed on leukemic cells from 3 patients initially, and from 6 patients after short term in vitro culture without stimulation. bcr rearrangements were not observed in 3 patients tested. RNA analysis showed that 5 patients expressed P190bcr-abl pattern and one patient expressed P210bcr-abl pattern using polymerase chain reaction study. We conclude that Ph1 positive acute leukemia had a poor prognosis and differentiate into both myeloid and lymphoid lineages as well as chronic myelogenous leukemia (CML), and that this disease could not be possibly distinguished from CML by use of the molecular studies.     

CD5+, CD7+, and CD19+ non-Hodgkin's lymphoma in a child]

The 9-year-old boy was admitted to Shizuoka Children's Hospital because of cervical lymphoadenopathy. Complete blood count showed normal RBC and platelet counts. WBC was 2700/microliters with no tumor cells. Bone marrow aspirate showed normocellularity with 34% tumor cells. Lymph node biopsy from his right neck was performed and the patient was diagnosed as non-Hodgkin's lymphoma (lymphoblastic type). Surface marker analysis disclosed that the tumor cells were positive for CD5, CD7, CD19, CD38, CD71, and Ia antigen. Chromosomal analysis of the cervical lymph node revealed 46, XY, t(7;14) (p15;q32). Molecular investigation with appropriate probe showed germ-line configurations of IgH gene, TcR beta gene, and TcR gamma gene, and one rearranged band of TcR delta gene. Monoclonality of tumor cells was demonstrated from chromosomal analysis and molecular study. CD7 and CD19 are not lineage specific antigens because CD7 is expressed on immature AML cells and CD19 is expressed on T ALL cells or AML cells. Moreover, TcR delta rearrangement is considered to occur at early phase of hematolymphoid cells. Based on these data, tumor cells of this patient is considered to originate from immature lymphoid cell, so-called lymphoid stem cell.     

Clinical features of latent dilated cardiomyopathy]

To identify the evidence of presymptomatic manifestations of dilated cardiomyopathy (DCM), we studied 30 patients with latent DCM (mean 37 +/- 14 years) who satisfied the following criteria: 1) left ventricular (LV) systolic function was slightly reduced; 2) LV end-diastolic dimension (< 54 mm) and coronary arteries were normal. The incidence of ECG abnormalities was relatively high; nonspecific ST-T changes were the most common (90%). The incidence of grade 3 or 4 ventricular premature contractions according to Lown's classification on 24 hour ambulatory ECGs was 50%. Perfusion defects were observed in 83% on the thallium-201 images. Right ventricular biopsy showed that the interstitial fibrosis was milder (9 +/- 9%) in the study subjects than in 32 patients with DCM (17 +/- 8%) who were treated in our hospital, but there were no significant differences in the diameters of the myofibers (15 +/- 4 vs 17 +/- 4 microns) between the 2 groups. During the follow-up study, deterioration of LV dysfunction was observed in 3 patients. One patient died suddenly. These findings proved the importance of the early detection and characterization of latent state of DCM.     

Clinical features of Q fever pneumonia]

We report four cases of Q fever pneumonia diagnosed using PanBio Coxilla burnetii ELISA. The patients, a 21-year-old woman, a 53-year-old man, a 74-year-old man and a 87-year-old man, were among 284 with community-acquired pneumonia who were treated as inpatients from March 2001 till March 2003. The frequency of Q fever pneumonia in community-acquired pneumonia was 1.4%. The 21-year-old woman was a typical case of Q fever pneumonia, since her clinical features showed 1. the breeding of cats, 2. development from a fever and non-productive caught in March, 3. multiple soft consolidations in the chest radiograph, 4. normal WBC count, 5. cure by administration of clarithromycin. The pneumonias of the other 3 cases were considered to be mixed infections, with bacteria such as Streptococcus pneumoniae and Haemophilus influenzae. Their clinical features were 1. elderly male patients with underlying diseases, 2. development from fever and cough with purulent sputum in winter, 3. coarse crackle on auscultation, 4. consolidation with pleural effusion in chest radiograph, 5. leukocytosis, elevation of BUN, hyponatremia, 6. a few cases with unfavorable prognoses despite medication with carbapenem and minocycline. These findings suggested that two types of pneumonia exist; one with the usual features of atypical pneumonia, and the other presenting the clinical features of bacterial pneumonia of the elderly due to a mixed infection including C. burnetti.     

抗核抗体阳性肝硬化患者的临床特点

为了探讨抗核抗体 (ANA)阳性肝硬化患者的临床特点及环磷酰胺的治疗效果 ,检测 82例肝硬化患者血清ANA ,比较ANA阴性和阳性肝硬化患者的临床特点 ,观察环磷酰胺治疗ANA阳性肝硬化患者的疗效。结果与ANA阴性肝硬化患者比较 ,ANA阳性肝硬化患者女性多见 ,发病年龄较大 ,乙型肝炎病毒感染率低 ,Child -Pugh分级C级比例高 ;腹水、下肢浮肿症状较重 ;血清白蛋白水平低、球蛋白水平高 (P <0 0 5 )。ANA阳性肝硬化患者经环磷酰胺治疗后症状均好转 ,其中与对照组比较 ,腹水、下肢浮肿症状改善明显 (P <0 0 5 ) ;血清总蛋白、白蛋白水平增加 ,球蛋白水平下降 (P <0 0 5 ) ;部分患者ANA转阴 (P <0 0 5 )。说明ANA阳性肝硬化患者有其自身的临床特点 ,环磷酰胺治疗有效。     

Clinical features of idiopathic mitral valve prolapse]

This paper ask a question; is there a generalized defect of the connective tissue among subjects with primary mitral valve prolapse? Based on clinical methods it was found that paired subjects with and without primary mitral valve prolapse, are different in respect of an arbitrary score of joint hypermobility and phenotypic features. This is a statistically significant difference and suggest a possible molecular defect affecting connective tissue in people with primary mitral valve prolapse. Therefore, biochemical and molecular studies should be done to further characterize this abnormality.