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C Devalck

Publications and source records attributed to C Devalck.

30 records · Page 2Linked to original sources

Transplanted sickle-cell disease patients with autologous bone marrow recovery after graft failure develop increased levels of fetal haemoglobin which corrects disease severity.

Bone marrow transplantation (BMT) is the only curative therapy for sickle-cell disease (SCD), but is not devoid of failure risk. Nine patients with severe SCD were grafted in our institution between 1988 and 1993. Six patients successfully engrafted, but three failed to engraft and had delayed autologous recovery. All patients had, prior to BMT, low levels of fetal haemoglobin (HbF < or = 3.5%). No change in HbF occurred in successfully grafted patients. In the three patients with graft failure HbF increased and remained persistently present at a high level (> or = 22%) 14 months, 16 months and 39 months post BMT, although two of the three patients were homozygous for either the Benin or the Central African Republic haplotype, a characteristic associated with low HbF level. Of interest, these three previously severely affected patients remain free of vaso-occlusive events. The mechanism responsible for the expression of high levels of HbF in our three patients with graft failure is not understood, but it protects them from the recurrence of severe vaso-occlusive crises.

Anemia, Sickle Cell↗

Anaplastic large cell lymphoma of true histiocytic origin in an infant: unusual clinical, hematological, and cytogenetic features.

We report here the case of a 7-month-old boy who developed anaplastic large cell lymphoma of true histiocytic origin or malignant histiocytosis, with fever, bone and bone marrow infiltration. Usual clinical features were absent. The neoplastic nature of the disease was supported by the presence of clonal chromosomal abnormalities [t(6;8)(p23;p21),der(8)del(8)(q11aq13), der(22) t(11;22) (q13;13)]. Neither B nor T lineage could be demonstrated here. Morphology, ultrastructural analysis, surface antigens expression, and cytogenetics were more specific for the monocyte-macrophage lineage.

Bone Marrow↗

Long-term disease-free survival in a child with refractory metastatic malignant germ cell tumor treated by high-dose chemotherapy with autologous bone marrow rescues.

We report the case of a 8-month-old girl who presented with a metastatic malignant germ cell tumor (MGCT) that proved to be resistant to chemotherapy, including salvage platinum-based combination therapy, surgery, and metastatectomy. The child was then treated with high-dose carboplatin, etoposide, and cyclophosphamide with autologous bone marrow rescue (ABMR) for two courses, since the first course was well tolerated and resulted in serum alpha-f etoprotein normalisation. The child remains in complete remission 3 years post-ABMR and 4 years post-diagnosis, with no detectable treatment-related sequelae. Thus, as already reported in adults, dose-intensive chemotherapy with ABMR may have curative potential in children with refractory MGCT.

Antineoplastic Combined Chemotherapy Protocols↗

[Stomatococcus mucilaginosus meningitis in immunocompromised child].

BACKGROUND: Opportunistic infections are responsible for significant morbidity and mortality in patients with malignancy developing granulocytopenia as a result of therapy. A case of Stomatococcus mucilaginosus meningitis is reported. CASE REPORT: A 2 year-old boy was admitted because he had developed fever and neutropenia during chemotherapy given for neuroectodermal tumor. He was previously treated for a Stomatococcus mucilaginosus septicemia with vancomycin given for 15 days through an intravenous catheter which has subsequently been left in place. At admission, fever was associated with severe degree of aplasia (70 WBC/mm3). The patient was given IV ceftazidime plus amikacin. Two days later, the boy developed acute meningitis due to Stomatococcus mucilaginosus; he was then given IV vancomycin (40 mg/kg/d) and imipenem (100 mg/kg/d). Persistence of abnormal clinical and bacteriological findings required subsequent intrathecal administration of vancomycin (1.5 mg/d) for 5 days. CSF cultures were negative 2 days later, leading to stop IV antibiotics after 3 weeks. The catheter was removed 1 week later. CONCLUSION: This patient represents the third reported case of Stomatococcus mucilaginosus meningitis. Combined intrathecal and systemic administration of vancomycin seems to have been useful in our case.

Child, Preschool↗

Complete remission following donor leukocyte infusion in ALL relapsing after haploidentical bone marrow transplantation.

A 7-month-old boy with a high risk ALL harbouring the translocation (4;11) was grafted with an haploidentical bone marrow from paternal origin. At time of relapse, 11 months after BMT, he received donor leukocyte infusions (DLI) which put him in second CR. GVHD and pancytopenia occurred 2 weeks after DLI and were fully reversed with CsA + prednisolone. Six months later, the child continues to be in second CR, off steroid therapy, without any signs of GVHD. Our limited experience indicates that a second CR can be obtained with acceptable toxicity by DLI in very high risk ALL children who have been previously grafted with haploidentical bone marrow cells.

Bone Marrow Transplantation↗

Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia.

In sickle cell anemia (SCA), the loss of reticuloendothelial function is the result of vasoocclusive events occurring in the spleen. Such asplenia occurs early in the course of the disease and is considered to be permanent in late childhood. In this report, three patients 10, 11, and 14 years of age suffering from severe SCA and found to be asplenic were treated by bone marrow transplantation (BMT). Before transplantation, all three patients had loss of reticuloendothelial splenic function, as assessed by the presence of abundant Howell-Jolly bodies on blood smears and absence of technetium 99m (99mTc) splenic uptake. After BMT, Howell-Jolly bodies disappeared from blood smear, whereas 99mTc isotopic scan found normal isotope uptake. Our data indicate that BMT can correct "permanent asplenia" in SCA patients. However, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.

Adolescent↗

[Granulocytic sarcoma with mediastinal involvement].

BACKGROUND: Granulocytic sarcoma is more frequent in adults; it can be a tumoral localization of acute non-lymphoblastic leukemia. This report describes an infantile case revealing an acute myeloid leukemia. CASE REPORT: A 14 month-old girl was admitted because of enlarged bilateral cervical lymph nodes. They had increased in volume over the past month and were accompanied by fever and anorexia. The liver and spleen were moderately enlarged. Hematologic data were: Hb = 7.5 g%; leucocytes = 14,900/mm3; platelets = 614,000/mm3. There were no abnormal cells. X-rays and MRI showed a mediastinal mass. Bone scintigraphy was normal but the myelogram showed a few atypical cells that were also seen in a biopsy of the cervical lymph node. Electron microscopic examination of these cells showed that they were myeloid in origin. This was confirmed by immunohistochemistry. The cytogenetic examination showed many chromosomal abnormalities in the lymph node and myelogram. The child is now recovered after nine months of chemotherapy. CONCLUSION: Diagnosis of acute myeloblastic leukemia in children is difficult when it is revealed by granulocytic sarcoma without major infiltration of bone marrow. The clinical presentation as a mediastinal mass is rare. The cytogenetic study was determinant for diagnosis.

Bone Marrow↗

[Autologous bone marrow graft in solid tumors in childhood].

Curability in children suffering from malignant solid tumor is 50%. Thus, high dose chemotherapy with or without total body irradiation followed by autologous bone marrow transplantation (ABMT) has been proposed to patients suffering from cancer either at initial diagnosis (poor prognosis tumor) or at relapse. Thanks to these studies, drugs having dose effects properties have been selected. In some tumors, ABMT has significantly improved patients median survival. It remains to be determined if: 1. high dose chemotherapy protocols with ABMT are superior to new aggressive chemotherapeutic protocol without ABMT. 2. ABMT increases the curability of high risk patients.

Bone Marrow Transplantation↗

[Indications for bone marrow graft in severe hemoglobinopathies].

Allogenic bone marrow transplantation (ABMT) is the only curative approach for sickle cell anemia and major beta-thalassemia. In sickle cell anemia, ABMT can be proposed for severe clinical disease. In major beta-thalassemia, it must be proposed to young patients who have an HLA identical familial donor.

Anemia, Sickle Cell↗

[Bone marrow graft in hereditary diseases].

Since 1968, bone marrow transplantation became the first line therapy for selected metabolic and immunological hereditary disorders. Actually, advances in the supportive care in bone marrow transplantation and a better knowledge of the immunology of BMT complications has been associated with a better disease correction and an increase in long term survival. New approaches are under investigation and include: hematopoietic growth factors, enzymatic replacement and gene therapy. However at the present time BMT is still the only curative treatment for selected hereditary disorders.

Bone Marrow Transplantation↗