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H J Weh

Publications and source records attributed to H J Weh.

At least 37 records · Page 2Linked to original sources

[Treatment of thrombotic thrombocytopenic purpura (Moschcowitz's disease) with vincristine].

A 64-year old female had been diagnosed as having thrombotic thrombocytopenic purpura (TTP; Moschcowitz syndrome) in 1984. The initial episode and one recurrence 5 years later were successfully treated with fresh plasma transfusions without plasmapheresis. Two days before the present admission, she noticed pain in the knee and haematomas in both legs. Marked thrombocytopenia (9000/microliters), haemolytic anaemia (lactate dehydrogenase 1800 U/l, haemoglobin 7.8 g/dl, haptoglobin 5 mg/dl) and skin haemorrhages suggested a second recurrence of TTP. The blood film showed a massive increase in fragmented cells (more than 30 per field), microspherocytes and normoblasts. Thrombocyte values initially rose on daily treatment with plasmapheresis using membrane separation and fresh plasma exchange. However, after two attempts at stopping therapy, plasmapheresis and fresh plasma replacement failed to normalise thrombocyte values. The patient had a total of 44 plasmaphereses. Intravenous administration of three doses of 1.5 mg vincristine at intervals of 7 and 5 days combined with further plasmapheresis treatment soon led to normalisation of the thrombocyte count (320,000/microliters), a fall in lactate dehydrogenase (120 U/l) and a disappearance of fragment cells in the blood smear. The patient is still in complete remission 6 months after stopping treatment. Treatment with vincristine should be considered for TTP refractory to treatment.

Bone Marrow Examination↗

Infusion of tumor-contaminated bone marrow for autologous rescue after high-dose therapy leading to long-term remission in a patient with relapsed Philadelphia chromosome-positive acute lymphoblastic leukemia.

We report a female patient with relapsed Philadelphia chromosome-positive acute lymphoblastic leukemia remaining in long-term second remission after high-dose radiochemotherapy followed by autologous stem cell rescue with bone marrow showing evidence of residual disease. The Philadelphia chromosome and a positive signal for the BCR/ABL p185 translocation by the polymerase chain reaction were detected in the harvested marrow. After mafosfamide-purged marrow failed to engraft, her unpurged 'back-up' bone marrow was also infused. Control marrow examinations after recovery were repeatedly positive for the BCR/ABL translocation on PCR analysis turning negative 30 months after high-dose therapy. She remains in unsustained complete clinical remission for 48+ months showing no evidence of leukemia by cytological and cytogenetic analyses.

Adult↗

Laparoscopic splenectomy. Technique and results in a series of 27 cases.

Between early 1992 and December 1994, laparoscopic splenectomy was performed in 27 patients with idiopathic thrombocytopenia (ITP), hairy-cell leucemia, HIV, or Hodgkin's disease. In all cases medical treatment, especially cortisone therapy, failed. In Hodgkin's disease the splenectomy was combined with liver biopsies and dissection of parailiacal, paraaortic, and mesenteric lymph nodes for abdominal staging. The operation was performed using four trocars; the splenic vessels were divided by a linear stapler. In general the spleen was removed in a bag through a slightly enlarged trocar incision or after morcellation. Three patients needed a small laparotomy for the removal (laparoscopic assisted). In a recent case of Hodgkin's disease the intact spleen was removed via posterior colpotomy. In 22 of 27 cases (81%) the operation was finished laparoscopically. Five times a conversion to conventional laparotomy was necessary because of bleeding of enlarged lymph nodes at the hilum. Wound infections occurred in two cases. In one patient with ITP the platelet count did not improve and continuous blood loss led to relaparotomy at the 1st postoperative day. No surgical bleeding was found. All patients tolerated a fluid diet at the 1st postoperative day and hospitalization time was 4.4 days (range 3-14). Regarding the low complication rate and the advantages of a smaller abdominal trauma in the postoperative period, the laparoscopic approach for elective splenectomy and laparoscopic abdominal staging has a substantial benefit for the patients.

Adolescent↗

Correlations between karyotype and cytologic findings in multiple myeloma.

In multiple myeloma, correlations between cytogenetic and morphologic findings are hampered by the relatively scarce chromosomal data and the lack of a widely accepted morphologic classification. The aim of the analysis, comprising 111 patients with multiple myeloma, was to study possible correlations between karyotype and a morphologic classification proposed by Bartl et al. Grade of plasma cell infiltration, predominant cell types (Marschalko, small, cleaved, polymorphous, asynchronous, blastic) and grade of malignancy are the basis of this classification. A pathologic karyotype was found in the bone marrow of 39/111 patients (35%). The incidence of chromosomal anomalies closely correlated with the grade of infiltration, plasma cell type and grade of malignancy. Chromosomal anomalies were rarely detected in patients with low infiltration (16%), but they were frequently found in high-grade infiltration (69%). A low incidence was found in Marschalko (25%) or small cell type (15%); the incidence was much higher in cleaved (75%), asynchronous (65%) and basic cell types (71%). An abnormal karyotype was more frequently found in high (71%) than in intermediate (53%) or low (23%)-grade malignant multiple myeloma. The most consistent structural chromosomal aberration found in five patients was translocation t(11;14)(q13;q32). In four of the five patients small, often cleaved plasma cells were the predominant cell types. These reported correlations between morphological and cytogenetic findings must be confirmed by future studies.

Bone Marrow↗

Demonstration of i(17q) in metaphase and interphase of malignant hematopoietic cells by fluorescence in situ hybridization.

In 10 patients with various hematopoietic disorders, i(17q) has been studied by the technique of fluorescence in situ hybridization (FISH) both in metaphase and interphase cells using an alpha-satellite centromere-specific probe #17. We observed some minor quantitative differences between metaphase and interphase cells with regard to the incidence of i(17q) in individual cases and considerable heteromorphism of the size of signal of i(17q) among different cases. Our results suggest that formation of i(17q) results from a break in the p-arm proximal to the centromere and that the breakpoints vary from case to case.

Aneuploidy↗

Weekly therapy with folinic acid (FA) and high-dose 5-fluorouracil (5-FU) 24-hour infusion in pretreated patients with metastatic colorectal carcinoma. A multicenter study by the Association of Medical Oncology of the German Cancer Society (AIO).

BACKGROUND: No effective salvage therapy is known for patients with metastatic colorectal carcinoma progressive after chemotherapy with 5-fluorouracil (5-FU)/folinic acid (FA) or 5-FU/alpha interferon (IFN) combinations. The aim of the study was to test whether weekly FA/high-dose 5-FU is an effective therapy in pretreated patients with progressive metastatic colorectal carcinoma. PATIENTS AND METHODS: Between January and December 1992, 57 patients with metastatic colorectal carcinoma were treated with weekly infusions of high-dose 5-fluorouracil (5-FU) (2600 mg/m2 as 24-hour infusion) and folinic acid (FA) (500 mg/m2 as 1-hour infusion prior to 5-FU). All patients were pretreated with chemotherapy, most of them with regimens containing 5-FU i.v. bolus/FA or 5-FU/alfa interferon (IFN), and all had documented progressive disease at the time of entering the study. In patients with partial remission (PR) or stable disease (SD) with improvement of their clinical condition, therapy was continued until progressive disease (PD) was documented. In all other patients therapy was stopped after one course (6 infusions). RESULTS: 5/57 patients (9%) achieved PR, 32/57 (56%) SD, in 19/57 (33%) disease was progressive and one toxic death occurred. 26/32 patients (81%) with SD or PR after the first chemotherapy again obtained SD or PR on high-dose 5-FU/FA but only 8/18 (44%) of those with PD after first chemotherapy did so. The median duration of SD/PR was 3 months and the median survival for all patients 8 months (range 3-17+). Apart from one toxic death, toxicity consisting for the most part of mucositis (n = 24), nausea (n = 23), diarrhoea (n = 18) and hand-foot syndrome (n = 12) was moderate. CONCLUSION: Pretreated patients with metastatic colorectal carcinoma, notably those with a primary PR or SD, can probably benefit from weekly high-dose 5-FU/FA.

Adult↗

Weekly high-dose 5-fluorouracil and folinic acid as salvage treatment in advanced gastric cancer.

BACKGROUND: A weekly schedule of a 24-h high-dose 5-FU infusion in combination with folinic acid was investigated as salvage therapy in patients with advanced gastric cancer. PATIENTS AND METHODS: Seventeen patients with progressive disease after chemotherapy were treated with a weekly schedule of 500 mg folinic acid/sqm by i.v. 2-h infusion followed by an i.v. 24-h infusion of 2.6 g 5-FU/sqm times six. Fourteen of the 17 patients (82%) had been pretreated in regimens which included 5-FU/folinic acid i.v. bolus. RESULTS: Toxicity was mild, with diarrhea, mucositis and nausea; WHO grade III/IV diarrhea was observed in only two patients. Three patients achieved partial remissions (18%; 95% confidence interval: 0%-38%) and 7 patients stable disease (41%; 95% confidence interval: 17%-64%). Their median survival time was five months (range 1.5-10 months) after the onset of salvage therapy. CONCLUSIONS: This schedule of weekly 24-h infusions of high-dose 5-FU and folinic acid is effective, even in patients pretreated with or resistant to regimens including 5-FU/folinic acid i.v. bolus. Based on these data, it appears that this schedule of high-dose 5-FU/folinic acid in first-line combination chemotherapy merits investigation.

Adult↗

Immunophenotype of mitotic cells with clonal chromosome abnormalities demonstrating multilineage involvement in acute myeloid leukemia.

Eleven patients with acute myeloid leukemia (AML) were studied with a technique that simultaneously identifies cytogenetic abnormality and immunophenotype of the same mitotic cell. To determine the cell lineages with abnormal karyotypes, monoclonal antibodies in the alkaline phosphatase-antialkaline phosphatase (APAAP) detection procedure were used. The granulocytic/monocytic lineage was involved in the leukemic process in all 11 patients. In nine patients, we also detected abnormal karyotypes in the erythrocytic and/or megakaryocytic lineages. All four patients with secondary AML showed involvement of the granulocytic/monocytic, erythrocytic, and megakaryocytic lineages into the leukemic process, as compared with five of seven patients with de novo AML. One patient with trisomy 8 showed erythrocytic participation in the leukemic process, but in another the erythrocytic lineage had only normal karyotypes. Thus, in AML, the chromosome abnormalities apparently usually originate at the multipotent progenitor cell stage, since in addition to granulocytic/monocytic lineages, erythrocytic and/or megakaryocytic lineages were also involved. Some patients show involvement of granulocytic/monocytic lineages only, however, suggesting that the target cell belongs to a more mature committed progenitor cell stage.

Adult↗

[Staging laparoscopy in Hodgkin's disease. A valid alternative to staging laparotomy].

A 30-year-old man with Hodgkin's disease, stage IA, had received radiotherapy to the supradiaphragmatic lymph nodes two years ago. During follow-up observations the patient felt well, but sonography revealed three tumours in the spleen. The erythrocyte sedimentation rate was found to be raised to 50/75 mm. Therefore, splenectomy and staging of the other parts of the abdomen for histological clarification and deciding on further therapeutic action were indicated. Thus splenectomy with bilateral liver wedge resections were performed laparoscopically, together with excision of mesenteric, paraaortic and para-iliac lymph nodes. The patient was discharged home on the 4th postoperative day. Histological examination revealed stage IIIE Hodgkin's disease. This case demonstrates that in favourable conditions staging laparoscopy can obtain the same information as conventional staging laparotomy, but it is much better tolerated by the patient.

Adult↗

GCSF gene is expressed but not rearranged in a patient with isochromosome 17q positive acute nonlymphocytic leukemia.

We describe a patient with acute nonlymphocytic leukemia (ANLL) and isochromosome 17q as the sole cytogenetic abnormality. ANNL with i(17q) may represent a distinct entity with certain clinical features, such as male sex, hepatosplenomegaly, and characteristic findings in bone marrow (BM) cytology, including hypercellularity, marked basophilia and eosinophilia, and massive increase in abnormal megakaryocytes. Molecular studies of peripheral blood (PB) cells of our patient, by polymerase chain reaction (PCR) analysis, showed expression of the GCSF gene, which is located on 17q. Southern blots hybridized with a GCSF probe showed no rearrangement of this gene as has been described in some patients with i(17q) positive chronic myeloid leukemia (CML).

Blotting, Southern↗

Karyotype in multiple myeloma and plasma cell leukaemia.

Between October 1988 and October 1991, 104 patients with multiple myeloma and 6 with plasma cell leukaemia were studied cytogenetically. Abnormal karyotypes were found in bone marrow cells of 33 patients (30%). Most pathological karyotypes were complex with numerous modal and structural anomalies. Numerical anomalies most frequently involved chromosome 11 and structural aberrations occurred most often in chromosomes 1, 11 and 14. The most consistent structural aberration was a 14q+ chromosome (10 patients) resulting from a t(11;14)(q13;q32) in 4 patients and a t(8;14)(q24;q32) in 1 patient. Sequential cytogenetic studies were performed in 15 patients. In 5 of 8 cases with a normal karyotype at diagnosis, chromosomal anomalies were detected when disease progressed. In concomitant cytogenetic/cytological studies it was found that in the majority of patients with normal karyotype the mitoses originated from contaminating normal bone marrow cells. Pathological karyotypes were detected more frequently in pretreated than in untreated patients, in patients with plasma cell leukaemia than in patients with multiple myeloma, in patients with stage III and dense bone marrow infiltration than in patients with stage I. Patients with abnormal karyotype, irrespective if pretreated or not, had a significantly shorter median survival than those with normal karyotype. These findings suggest that karyotype is an independent prognostic factor in multiple myeloma.

Adult↗

Malignant haemangiopericytoma of the chest wall.

A patient with malignant haemangiopericytoma of the right second rib is described. En-bloc resection of this rare tumour with the adjacent upper lobe of the lung and part of the chest wall could be achieved. The clinical and morphologic features of this case are presented. On account of the high risk of local recurrence and distant metastasis, we think an adjuvant chemo- and radiotherapy is indicated.

Adult↗

Treatment results obtained in metastatic soft-tissue sarcoma with a combination of doxorubicin and dacarbazine or doxorubicin and ifosfamide.

Between 1982 and 1986, 38 patients with soft-tissue sarcomas were treated with the combination doxorubicin/dacarbazine (group A); between 1986 and 1990, another 46 patients received doxorubicin/ifosfamide (group B); and between 1990 and 1991, 11 patients received an alternating regimen of doxorubicin and ifosfamide (group C). The patients' characteristics were comparable among the three groups. Overall remission rates were 34% in group A, 44% in group B, and 18% in group C. The duration of remission was 10 months and the median survival was 13 months in groups A and B. Toxicity, especially myelotoxicity, was severe, with no marked differences being noted between the groups. We conclude that both doxorubicin/dacarbazine and doxorubicin/ifosfamide are active regimens in metastatic soft-tissue sarcomas; nevertheless, the overall prognosis for these patients remains poor.

Adolescent↗