摘要:

The bone marrow of an 11-year-old girl with cyclic neutropenia was examined by electron microscopy. In the nonneutropenic phase, mature polymorphonuclear neutrophils had numerous bundles of filamentous inclusions in both cytoplasms and nucleus. Primary granules were slightly increased in number. Similar filament-like inclusions also have been reported in some cases of hemopoietic dysplasia; however, our case showed no abnormality in chromosomes, no thrombocytopenia. and no leukemic change.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

A 16-year-old patient underwent partial gastrectomy for leiomyosarcoma of the stomach. Following resection, he received combination chemotherapy that included Adria-mycin and dimethyltriazenoimidazole carboxamide (DTIC). with the cumulative Adriamycin dose being 405 mg/m2. The patient was subsequently treated with vincristine, actinomycin D. and cyclophosphamide. Six hours after receiving his fourth dose of cyclophosphamide, the patient developed signs and symptoms of acute anterior wall myocardial infarction. Although he recovered from this initial cardiac event, he subsequently experienced several additional episodes of vascular occlusion involving the cerebral, femoral, coronary, and pulmonary arteries. Cardiac catheterization demonstrated all coronary arteries to be normal. Both ventricles were hypokinetic, and bilateral mural thrombi were demonstrated; these were the presumed source of the embolic phenomena. To our knowledge, this is the first description of repeated coronary artery embolization following cancer chemotherapy in a patient without evidence of preexisting cardiac abnormalities.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Neutrophilic eccrine hidradenitis is a rare but distinct dermatosis that has recently been described in adults with acute myelogenous leukemia. We report the first pediatric case in an 11-year-old girl with acute myelomono-cytic leukemia who developed this eruption following her first and second course of VAPA-10 induction chemotherapy. Skin biopsy revealed selective neutrophilic infiltration around the eccrine coils, which is characteristic of this dermatosis. The clinical and biopsy findings of our patient are presented and compared with the previous three reported cases.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

A level of circulating “pitted” or vesiculated red blood cells higher than 3.50 was recently reported in studies in the Cooperative Study of Sickle Cell Disease to correlate with splenic dysfunction as shown by spleen scans. Reversal of splenic dysfunction by transfusion in children with sickle cell anemia (SS disease) is known to occur in the young child. We report two older patients with homozygous sickle cell disease, aged 17 and 21 years, whose spleen function, as measured by pit count. was restored to normal range after transfusion.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Idiopathic thrombocytopenic purpura (ITP) of childhood is a disorder characterized by a history of previous viral illness followed by acute onset of low circulating platelet count with present or increased megakaryocytes in the bone marrow. The majority of children recover a normal platelet count within 6 months to 1 year after onset of the disease. To better understand the regulation of mega-karyocytopoiesis in this disorder, we studied nine patients with childhood ITP with the plasma clot colony assay in vitro for megakaryocyte colony forming units (CFU-Mk). Mononuclear bone marrow cells from some of the patients with ITP contained greater numbers of CFU-Mk and greater numbers of cells per colony than mononuclear bone marrow cells from healthy adult volunteers (p < 0.026) when the cultures contained no added megakaryocyte colony-stimulating activity (Mk-CSA). The serum from patients with ITP did not stimulate in vitro megakaryocytopoiesis from healthy adult volunteers' bone marrow mononuclear cells above baseline values. These findings are consistent with the hypothesis that a decrease in bone marrow magakaryocytes is needed for Mk-CSA production. Alternatively. Mk-CSA is consumed by active megakaryocytopoiesis in the bone marrow.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Patients with osteogenic sarcoma are at a very high risk of developing pulmonary metastatic disease. Bleomycin has recently been reported to cause pulmonary nodules mimicking metastatic disease. Our case adds what we believe to be the first report in the pediatric literature and describes new findings that we believe are so suggestive of bleomycin nodules that immediate biopsy to exclude metastatic disease may not be necessary.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Neutropenic enterocolitis (also termed typhlitis) is an acute necrotizing process involving segments of the large and small intestine that occurs in the setting of agranulocytosis, most commonly in patients with acute leukemia. Rapid diagnosis and treatment is necessary for survival. We present a patient in whom abdominal ultrasonography revealed evidence of bowel wall thickening and ascites. confirming the diagnosis of neutropenic enterocolitis. Sonography offers a rapid. safe, and noninvasive means of diagnosis for this condition.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Histiocytosis-X may involve many tissues, including the central nervous system. This report is the first documentation of histiocytosis-X occurring exclusively in the spinal cord. We present a patient with cervical spinal cord disease causing spastic paraplegia, flaccid right arm weakness, and sensory loss. Magnetic resonance imaging of the spinal cord clearly demonstrated the entire lesion. Following surgical decompression and biopsy, the cervical spinal cord was treated with low-dose radiation, which resulted in complete regression of the lesion by radiologic assessment. The child became neurologically normal, and histiocytosis-X has not been detected in other organ systems.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

The growth hormone (GH) responses to (A) GRF 1–44. 1 ug/kg i.v., (B) L-dopa and either arginine. insulin, or glucagon. and (C) exercise were evaluated in 10 children (3 girls. 7 boys: ages 10 years to 15 years. 8 months). 2–10.75 years following cranial irradiation for medulloblastoma (8 patients), pineoblastoma (1 patient), and a fourth ventricular ependymoma (I patient). Nine of the 10 children had abnormal growth rates. All children were euthyroid at the time of the study. The mean 0–60-min peak GH response to GRF (10.06 ± 2.6 ng/ml) in the patients was less than the mean peak GH response (29 ± 2.3 ng/ml) in the control children (n = 7). In 6 patients (5 with poor growth rates), a decreased GH response was noted to GRF and all other tests. Of the remaining patients, all with poor growth rates, two patients demonstrated an adequate response to GRF and pharmacologic testing: one patient had a normal GH response to GRF with a low GH response to pharmacologic testing: and one patient had a low response to GRF. despite a normal response to both exercise and pharmacologic testing. The decrease in mean peak GH response to, GRF in the patient population confirms that radiation to the hypotha-lamic-pituitary region produces abnormalities in growth hormone release. Furthermore, in these patients, discordant GH responses to GRF and pharmacologic or physiologic tests can be observed. The abnormality in growth hormone release may result from a hypothalamic dysfunction in GRF release and/or damage to GH secretory pituicytes.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

Two cases of infants with congenital leukemia who had severe, refractory hypertensive reactions to teniposide (VM-26) are described. Patients on a 5 mg/kg twice weekly schedule of teniposide had hypertensive reactions in which their systolic blood pressure was greater than 200 mm Hg after the second dose of teniposide. Hypertension combined with myelosuppression resulted in the patient's death in one case. Although the exact mechanism of this unusual toxicity of teniposide remains unknown, it might be an age-specific problem, considering the very young age of our patients. Meticulous monitoring of vital signs, including blood pressure, is mandatory in leukemic infants receiving teniposide.

ISSN:0192-8562    

出版商:OVID    

年代:1987

数据来源: OVID