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F Delacrétaz

Publications and source records attributed to F Delacrétaz.

At least 19 recordsLinked to original sources

Prognostic value of follicular dendritic cells in nodular sclerosing Hodgkin's disease.

AIMS: In nodular sclerosing Hodgkin's disease (NSHD), the prognostic relevance of the histopathological grading in two subtypes NSI (low-grade) and NSII (high-grade) remains controversial. Analysis of follicular dendritic cells (FDC) may provide new prognostic parameters. METHODS AND RESULTS: Tumours from 59 patients with NSHD were studied. Mean follow-up time was 8 years. Forty-one cases were classified as NSI and 18 as NSII. FDC were immunostained with the paraffin-resistant monoclonal antibodies CD21 and CNA.42. We distinguished three patterns in the neoplastic tissue: FDC1, the presence of well-defined follicle-like structures (n = 20); FDC2, the presence of largely destroyed FDC networks (n = 25); and FDC3, no or a few isolated FDC (n = 14). The three groups differed clearly regarding the frequency of relapse and the survival. The longest survival was seen in the FDC1 group, the shortest in the FDC3 group, the FDC2 group being intermediate (P = 0.0025). FDC status was a discriminating prognostic factor for all patients, and within various age and stage categories. Combining the FDC status and the NSI-NSII grading defined the best survival group as FDC1-NSI. CONCLUSIONS: Assessment of FDC pattern, associated with histological subtyping, brings valuable data for predicting survival and outcome in NSHD.

Adolescent

Systemic mastocytosis following a malignant ovarian germ cell tumour.

Cases of mediastinal germ cell tumours associated with haematological disorders (two cases of systemic mastocytosis included) have been reported previously. This combination is more frequent than would be expected by chance alone. We report the case of a 30-year-old woman, who presented with a systemic mastocytosis following a malignant ovarian germ cell tumour which was treated by chemo- and radiotherapy. The patient predominantly complained of skeletal pains, which led to an erroneous radiological diagnosis of fibrous dysplasia for years. An aggressive variant of systemic mastocytosis was diagnosed on bone marrow examination. Systemic mastocytosis was confirmed by splenectomy, liver biopsy and finally autopsy. The present case is unique because of the ovarian location of the germ cell tumour. We suggest our observation could be related to the broad group of haematological malignancies associated with germ cell tumours.

Adolescent

[Polyarthralgia, fever and purpura in a patient known to have splenomegaly].

A 43-year-old patient was admitted for swollen and painful ankles, knees and elbows, palpable purpura of the lower extremities and fever. Splenomegaly had been diagnosed 5 years previously. At clinical workup, spleen and liver size were increased and purplish papular skin lesions were noted above the ankles. Erythrocyte sedimentation rate was 2 mm/h. ASAT and ALAT as well as rheumatoid factor were moderately elevated. Serology was positive for hepatitis C. Differential diagnosis of palpable purpura and complementary diagnostic procedures are discussed.

Adult

Distribution and localization of Epstein-Barr virus subtypes A and B in AIDS-related lymphomas and lymphatic tissue of HIV-positive patients.

Tissue samples of 21 HIV-positive patients have been studied for the presence and distribution of Epstein-Barr virus (EBV) subtypes A and B. This was done by PCR, EBER in situ hybridization, and immunohistochemical detection of EBV latent membrane protein (LMP) in AIDS-associated malignant lymphomas (16 cases) and lymphatic organs of patients without lymphoma (5 cases). Eleven cases were considered to be EBV-positive, with type A in four, and type B virus in four other cases. In patients with malignant lymphoma, EBV was localized in tumour tissue exclusively. One patient without lymphoma presented with multiple EBV genome type B-positive cells in all the lymphoid tissue samples examined. In HIV-positive patients, both subtypes of EBV, A and B, may play a role in the pathogenesis of lymphoproliferative lesions.

Adult

Current concepts in pediatric burn care: management of burn wounds with cultured epidermal autografts.

Our experience with CEA is based on 21 patients operated on from 1986 to 1991. The areas covered with CEA measured 500 cm2 to 3160 cm2. At one setting no more than 40 sheets of 40 cm2 CEA have been transplanted. The take of CEA is over 75% when applied to dermis. The same holds true when covering "deepithelialised" skin homografts on immunosuppressed patients. Scar formation has not been a problem, and the overall results have been good.

Adolescent

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient.

We report a case of a 31-year-old HIV infected black female, who presented with asymptomatic generalized lymphadenopathy. Three particularly enlarged lymph nodes were biopsied (2 cervical and 1 axillary). The histological picture was consistent with a diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease. Large histiocytes, positive for a variety of macrophage markers and for the S-100 protein, were observed in the distended sinuses. A few hyperplastic follicles, such as usually seen in HIV-infection-associated lymphadenopathy, were present at the periphery of one lymph node. No infections agent besides HIV could be detected by histological or microbiological analysis or by in situ hybridization. This is the first reported case of SHML associated with HIV infection. The possible relationship between the two diseases is discussed.

Adult

Dysmegakaryopoiesis predicting response to therapy in acute myeloid leukaemia. A histologic and clinical study.

With standard induction therapy between 50 to 85% of patients with Acute Myeloid Leukaemia (AML) achieve Complete Remission (CR). We investigated whether any morphological feature of bone marrow (BM) plastic embedded biopsies could predict failure of therapy. We reviewed BM plastic embedded biopsies from 54 adult patients presenting with untreated AML. The main histologic parameters analysed were cellularity, dysmegakaryopoiesis (DysM), percentage of marrow blasts and fibrosis. CR was obtained in 34 of 49 treated patients (69%). The rate of CR was significantly lower in the group of patients presenting with DysM: CR was achieved in 54% of the 28 treated patients with DysM and in 90% of the 21 treated patients without DysM (p less than 0.02). Patients with DysM had a significantly lower blood count and bone marrow blasts at presentation. Median age was not significantly different in the 2 groups. Cellularity and fibrosis were not predictive. DysM may be the hallmark of an AML subgroup with distinct clinical behaviour and lower rate of CR with conventional therapy. DysM should be carefully looked for on BM marrow biopsies and aspirate from AML patients at diagnosis.

Acute Disease

Genotypic and phenotypic evidence of T-cell leukemia in a patient successfully treated by interferon-alpha for typical hairy cell leukemia.

A 53-year-old man was diagnosed to have typical hairy cell leukemia. Immunophenotyping of frozen splenic tissue showed clonality of hairy cells for mu lambda, confirmed by the corresponding immunoglobulin gene rearrangements. The patient was successfully treated with interferon-alpha (IF-alpha). In the fifth year of treatment with IF-alpha the morphology of peripheral blood mononuclear cells (PBMC) and of bone marrow infiltration changed with the appearance of numerous small to intermediate shaped lymphocytes of a T-helper phenotype. Frank leukemia, resistant to IF-alpha treatment and ultimately aggressive chemotherapy, developed. Emergence of this second clonal disease was confirmed by rearrangement studies performed on PBMC; rearrangements of both alleles of the TCR beta were identified, whereas the JH and lambda IVS genes were in germline configuration. The outgrowth of a second, malignant T-cell clone paralleled by the disappearance (down-regulation?) of the initial B-cell clone while under cytokine treatment is consistent with the possibility that IF-alpha favoured the emergence of this second clone.

Bone Marrow

[Refractory anasarca].

In a 68-year-old male hospitalized for a traffic accident physical examination revealed a left tibial fracture and an asymptomatic left pleural effusion. The evolution was characterized essentially by the appearance of oedema of the legs, ascites, and aggravation of the left pleural effusion with concomitant right effusion. Immediate therapy consisted of digitalis and diuretics. Several days later the patient suddenly complained of epigastric pain irradiating to his arms, followed by vomiting. ECG showed transitory elevation of ST segment in V3 to V5. Blood levels of CK and CK-MB were normal. An electrocardiogram showed extensive antero-septal-apical akinesis and a very small cardiac effusion. Pleural and abdominal tap revealed the presence of a milky liquid containing 40 g/l proteins, 8.6 mmol/l triglycerides and 2.5 mmol/l cholesterol, with no atypical or tumorous cells. CAT scan revealed the presence of a retroperitoneal mass extending from the diaphragm to the iliac crests and apparently enclosing the aorta and the vena cava and causing bilateral hydronephrosis. The development of chylous ascites and/or chylothorax indicates the presence of an extrinsic or intrinsic obstruction of lymphatic drainage or the existence of a laceration or rupture of the thoracic canal. Chylous ascites is not a frequent finding, since only 28 cases have been diagnosed in 20 years at the Massachusetts General Hospital and 71 in 30 years at the Mayo Clinic. In these 2 series a tumoral etiology was found in more than 85% of the cases. The most frequently encountered tumor was lymphoma followed by cancer of the pancreas, stomach, or other tumors. CLINICAL DIAGNOSIS: Retroperitoneal tumor, most probably lymphoma; inferior vena cava compression syndrome; subacute antero-septal infarction. In spite of the poor general condition of the patient exploratory laparotomy was performed, and several specimens were taken from the lymphatic nodes located at the angle of Treitz, in the mesentery and the mesocolon and along the celiac axis. HISTOPATHOLOGICAL DIAGNOSIS: Non-Hodgkin B-cell malignant lymphoma, low-grade, lymphoplasmacytoid (immunocytoma) according to the Kiel classification, or small lymphocytic, plasmacytoid according to the International Working Formulation (abdominal lymph nodes, liver, epiploon, peritoneum, rectus abdominis muscle). EVOLUTION: Chemotherapy was instored consisting of a combination of prednisone, vincristine, and cyclophosphamide. The patient's condition was complicated by peritonitis. Laparotomy revealed phlegmonous cholecystitis and a perforated colon. The post-operative course was characterized by septic shock, cardiac and respiratory insufficiency which resulted in the death of the patient. Autopsy confirmed the presence of a low-grade lymphoma stage IV (retroperitoneum, mesentery, cervical, axillary and abdominal lymph nodes, liver, spleen, pancreas, colon, rectum, kidneys, adrenal glands, bronchi, epicardium). No evidence of an infarctus or oth

Aged

[Therapy of thrombocytosis in myeloproliferative syndromes using recombinant interferon-alpha-2a].

In a prospective open study 16 consecutive patients with a myeloproliferative syndrome and thrombocytosis were treated with interferon (IFN) alpha-2a. 4 patients had polycythemia vera, 4 essential thrombocythemia, 3 myeloid metaplasia and 5 chronic granulocytic leukemia. Platelet counts decreased in all treated patients within 2 to 12 weeks from a median value of 1010 x 10(9)/l to 350 x 10(9)/l. No primary or secondary resistance was observed. The initial dose of IFN was 9 m U per day. After correction of the thrombocytosis, it was progressively reduced to a minimum dose of 3 m U per week. Despite the good platelet response to IFN, leukocytosis persisted in 3 patients and polycythemia in a further 3. Side effects and poor compliance required discontinuation of therapy in 6 patients. Special attention is focused on the follow-up in 6 patients who have been treated for more than 15 months.

Adult

[Syncope of undetermined origin].

A 48-year-old man with no significant medical history suddenly suffered, when walking, a sensation of thoracic pressure and severe dyspnea, soon followed by loss of consciousness without seizure. On hospitalization the following abnormal features were found: moderate cyanosis and cutaneous erythematous lesions; a blood gas analysis with moderate hypoxemia and slight compensated metabolic acidosis; and on chest X-ray a small band of atelectasis of the left lower lobe. An ECG, a continuous 24 hours Holter ECG recording and pulmonary scintigraphy were within normal limits. During the next few days the patient had several syncopal attacks without detectable cause. Two biopsies were then performed which established the diagnosis.

Autonomic Nervous System Diseases

[Retrospective study of 30 cases of myelodysplastic syndrome according to the French-American-British classification].

The bone marrow smears and marrow biopsies of 30 unselected consecutive patients with a myelodysplastic syndrome have been classified retrospectively according to the proposals of the French-American-British cooperative group (FAB). The diagnoses according to the FAB classification were refractory anemia (RA) in 3, RA with ring sideroblasts (RAS) in 5, RA with excess of blasts (RAEB) in 14, REAB in transformation (RAEB-t) in 5, and chronic myelomonocytic leukemia (CMML) in 3. The group comprised 19 men and 11 women with a median age of 68 years. Of 22 patients, 9 died after progression to acute non lymphoblastic leukemia (ANLL), 7 from infections, 2 from bleeding and 4 from unrelated causes. Actuarial survival is 10.5 months, 8 patients being at risk at the time of evaluation. Although the small number of patients does not allow statistical evaluation, our results are similar to those in the literature: prognosis is less good with an increasing number of marrow blasts and degree of cytopenia, and the risk of progression to ANLL is higher for REAB-t and REAB patients. Of 4 patients treated with small doses of cytosine-arabinoside (Ara-C), one responded and remained stable without treatment for 7 months. 2 patients resistant to small-dose Ara-C showed complete response to subsequent high-dose Ara-C treatment.

Aged

Histopathology of bone marrow in human immunodeficiency virus infection.

The clinical and haematological findings in 18 patients with human immunodeficiency virus (HIV) infection were correlated with the histological features of plastic embedded bone marrow biopsies. Fifteen patients presented with peripheral cytopaenia of one or several cell lines. Twelve (66%) of the 18 patients exhibited bone marrow findings including normo- to hypercellularity, myelodysplasia, lymphocytosis with or without plasmacytosis and fibrosis of the reticulin type. Seventeen patients had myelodysplastic features, 5 of the 3 haematopoietic lines, 10 of 2 lines and 2 of 1 line. Dysmegakaryocytopoiesis and dyserythropoiesis, seen in 88% and 83% of the 18 patients respectively, were the most common myelodysplastic features. Bone marrow gelatinous transformation (serous atrophy) was a conspicuous finding in 7 (38%) of the 18 patients. The constellation of histological features here described, although not pathognomonic, is highly suggestive of HIV infection. The pathogenesis of the haematological abnormalities in HIV infection is discussed.

Acquired Immunodeficiency Syndrome

Histopathology of myelodysplastic syndromes. The FAB classification (proposals) applied to bone marrow biopsy.

Twenty-eight cases of myelodysplastic syndrome (MDS) were reviewed to evaluate whether the morphologic criteria proposed by the French-American-British (FAB) Cooperative Group for marrow smears could be applied to glycol methacrylate embedded trephine biopsies. Bone marrow biopsies and marrow smears were examined separately and then compared for the following parameters: percentage of blasts, dyserythropoiesis, ring sideroblasts, dysmegakaryopoiesis, dysgranulopoiesis, monocytes, cellularity, fibrosis, and "abnormal localization of immature precursors". The results of the histologic (biopsies) and cytologic (marrow smears) examinations were in good agreement in 24 of 28 cases. The authors' results suggest that the five MDS types proposed by the FAB group can be reliably distinguished on bone marrow biopsy with knowledge of the peripheral blood blast and monocyte counts. When the bone marrow aspiration is inadequate, the biopsy can establish diagnosis and type of MDS and rules out aplasia or tumor infiltration as possible alternative causes of cytopenia.

Adult