Myeloid leukemia and myelodysplastic syndrome relapsing as granulocytic sarcoma (chloroma) after allogeneic bone marrow transplantation

Of 229 consecutive patients receiving allogeneic blood or bone marrow stem cell transplants for acute myeloid leukemia, chronic myeloid leukemia, or myelodysplastic syndrome between 1974 and 1996, 52 patients relapsed. The original tumor recurred as granulocytic sarcoma (chloroma) in three patients (1.3%). Chloroma was found in the ovary in two patients and in the central nervous system in one patient. None of these three patients had experienced > or = grade II acute or more than limited chronic graft-versus-host disease. The intervals between transplantation and recurrence with chloroma were 2, 6, and 13 years. Two patients received a second transplant, and all three died of treatment sequelae.     

Granulocytic sarcoma presented as a reactivation of chronic myeloid leukemia after allogenic marrow transplantation

The authors report the case of a chronic myeloid leukemia (CML) patient submitted to allogenic bone marrow transplantation, who had probably never entered complete remission. The disease was reactivated as a granulocytic sarcoma, next to a platinum plate installed to correct a tibia fracture 11 years earlier. Its final event was a myeloid Ph1 + blastic crisis that was unsuccessfully treated with high doses of sc interferon and citarabine.     

Kaposi's sarcoma (author's transl)

Kaposi's sarcoma of the skin and an aplastic syndrome occurred together in a 51-year-old patient. Macroscopically livid papules and nodules were observed. Histomorphologically endotheliomatous cell proliferation with signs of infiltrative growth was found. Because of the aplastic pancytopenia cytostatic treatment of the Kaposi sarcoma was contraindicated. The patient finally died of vascular failure with haemorrhagic diathesis being manifest. Syntropy of Kaposi's sarcoma with malignant haematological diseases is known. However, association with aplastic anaemia has not been observed so far. The pathogenesis of Kaposi's sarcoma is unknown.     

Granulocytic sarcoma as isolated extramedullary relapse after donor lymphocyte infusion in a patient with CML who relapsed after allogeneic bone marrow transplantation: a case report

Nineteen patients with spina bifida and bilateral dislocation of the hips were studied with a minimal follow-up of 10 years. The average age at review was 21 years (range, 10-31). Ten patients had an upper neurologic level (thoracic to L3), and nine had a low lesion (L4 to sacrum). Three patients had no hip surgery. A closed or open reduction was performed in 12 hips, supplemented by one or more surgical procedures. Of these, 10 remained enlocated, and two had redislocated. In all other hips, several surgical procedures were performed, aimed at improvement of hip-flexion deformity or stability. At follow-up, one patient had occasional pain in one hip, which was dysplastic. Of the 10 patients with a high lesion, only two were walkers, but both had bilateral dislocation of the hips at follow-up. In contrast, all nine patients were walkers, but four of these had bilateral dislocation at follow-up. We found that a level pelvis and good range of motion of the hips are more important for ambulation than is reduction of bilateral hip dislocation.     

Radiogenic sarcoma of the thyroid (author's transl)

A sarcoma in the region of the thyroid, observed 30 years after radiotherapy in a patient with syringomyelia is presented. Even after low dose radiation in childhood and adolescence tumours of the thyroid frequently can be diagnosed. They rarely occur in adults, because apparently of a long latent period. This case is thought not to be a specific thyroid tumour.     

Tissue hypercarbic acidosis as a marker of acute circulatory failure (shock)

Measurement of pH of the stomach wall (gastric intramural pH) by the tonometric method has been utilized both experimentally and clinically as an indicator of the capability of the stomach to extract and utilize oxygen. As such, it serves as a metabolic marker of acute perfusion failure (circulatory shock). More recently, researchers have found that increases in the PCO2 accounted for the decline in pH; this was documented in tissues other than the stomach wall, including the esophageal and sublingual mucosa. In this review, tissue PCO2 is identified as a universal indicator of impaired perfusion and contrasted with conventional hemodynamic and metabolic markers of perfusion failure.     

Prevalence of intracranial lesions in children initially diagnosed with disconjugate nystagmus (spasmus nutans)

A small number of children who develop disconjugate nystagmus, torticollis, and head titubation (spasmus nutans) have been found to have optic chiasm or third ventricle gliomas. However, the prevalence of glioma or other developmental abnormalities in this disorder is unknown because no large series of spasmus nutans cases has previously been reported. A reviewer of the records of 67 consecutive children initially diagnosed with spasmus nutans and followed for an average of 3.3 years at the St Louis Children's Hospital revealed the following: 61% had a history of prematurity, developmental delay, or other systemic abnormality; strabismus, most commonly infantile esotropia, developed in 55%; 43% had neuroimaging studies; and 0% had evidence of a glioma or showed signs of tumor on follow-up examinations. From this consecutive patient series, we estimate the prevalence of tumor in spasmus nutans to be less than 1.4%. Without other evidence of an intracranial mass lesion, neuroimaging of infants initially diagnosed with spasmus nutans may not be immediately warranted.     

N(N)-nicotinic blockade as an acute human model of autonomic failure

Pure autonomic failure has been conceptualized as deficient sympathetic and parasympathetic innervation. Several recent observations in chronic autonomic failure, however, cannot be explained simply by loss of autonomic innervation, at least according to our current understanding. To simulate acute autonomic failure, we blocked N(N)-nicotinic receptors with intravenous trimethaphan (6+/-0.4 mg/min) in 7 healthy subjects (4 men, 3 women, aged 32+/-3 years, 68+/-4 kg, 171+/-5 cm). N(N)-Nicotinic receptor blockade resulted in near-complete interruption of sympathetic and parasympathetic efferents as indicated by a battery of autonomic function tests. With trimethaphan, small postural changes from the horizontal were associated with significant blood pressure changes without compensatory changes in heart rate. Gastrointestinal motility, pupillary function, saliva production, and tearing were profoundly suppressed with trimethaphan. Plasma norepinephrine level decreased from 1.1+/-0.12 nmol/L (180+/-20 pg/mL) at baseline to 0.23+/-0.05 nmol/L (39+/-8 pg/mL) with trimethaphan (P<.001). There was a more than 16-fold increase in plasma vasopressin (P<.01) and no change in plasma renin activity. We conclude that blockade of N(N)-cholinergic receptors is useful to simulate the hemodynamic alterations of acute autonomic failure in humans. The loss of function with acute N(N)-cholinergic blockade is more complete than in most cases of chronic autonomic failure. This difference may be exploited to elucidate the contributions of acute denervation and chronic adaptation to the pathophysiology of autonomic failure. N(N)-Cholinergic blockade may also be applied to study human cardiovascular physiology and pharmacology in the absence of confounding baroreflexes.     

The common marmoset (Callithrix jacchus) as a model for neuroleptic-induced acute dystonia

To examine whether acute dystonia is induced by neuroleptic treatment, common marmosets were treated with haloperidol orally twice a week over 25 weeks until dystonic behavior was elicited. Movement disorders such as acute dystonia were observed 6 weeks after the initial treatment, and had appeared in all treated animals by 25 weeks. Once these movement disorders were induced, they consistently reappeared after further treatment with haloperidol, and once haloperidol dosing was discontinued, the episodes vanished. Then, various neuroleptic drugs (bromperidol, chlorpromazine, risperidone thioridazine, sulpiride, tiapride, and clozapine) or a nonneuroleptic drug (diazepam) were administered orally instead of haloperidol in the above animals. All the neuroleptic drugs except for clozapine elicited similar abnormal behavior, while diazepam failed to induce any dystonia. An anticholinergic drug, trihexyphenidyl, which is known to reduce acute dystonia in patients, was also given orally to the above haloperidol-sensitized animals, followed by further treatment with haloperidol 30 min later. This clearly suppressed the induction of dystonia by haloperidol. The similarity between these findings for haloperidol-pretreated common marmosets and clinical findings suggests that the present model is useful for predicting the potential of antipsychotics to induce acute dystonia in humans.     

Percutaneous transluminal coronary angioplasty (PTCA) as primary therapy in acute myocardial infarct

BASIC PROBLEM AND OBJECTIVE: Percutaneous transluminal coronary angioplasty (PTCA) is being increasingly considered as an alternative to thrombolytic treatment of acute myocardial infarction. Studies performed so far, some on selected groups of patients, have produced high initial results of success. This prospective study was undertaken to determined primary success, complications and recurrence after primary PTCA in acute myocardial infarction (AMI). PATIENTS AND METHODS: Primary treatment in the form of immediate PTCA of the infarct vessel was undertaken in 111 patients (84 men, 27 women; mean age 58.6 +/- 10.3 years) with AMI. PTCA was judged successful if the infarct vessel had been reopened to perfusion grade 3 and restenosis was < 50%. No thrombolytic treatment was given, but heparin infusions were given during and for 24-48 hours after the procedure. 13 patients (11.7%) were in cardiogenic shock or required cardiopulmonary resuscitation for infarct-related arrhythmias. RESULTS: The primary success rate of PTCA for the whole group was 91% (101 of 111 patients), but only 77% (ten of 13) among patients in cardiogenic shock and (or) after resuscitation. Acute re-occlusion (0-6 days after PTCA) occurred in seven patients. Eight patients (7.2%) died during the hospital phase (0-4 weeks), seven of whom had been in shock or required resuscitation (death rate 54%). The overall complication rate of the intervention was 6.3%. No emergency aortocoronary bypass was necessary. Repeat coronary angiography was performed in 71 of the 101 successfully treated patients 6 or 12 weeks after the PTCA. Re-occlusion was demonstrated in four (5.6%), a restenosis of more than 50% in 25% of patients. Mean left ventricular ejection fraction, obtained by planimetry from the levocardiogram was 58.6 +/- 9.3%. CONCLUSION: PTCA, performed immediately after acute myocardial infarction is an effective therapeutic measure with a high primary success rate.     

Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma

We report the case of a hepatic undifferentiated (embryonal) sarcoma (UES) arising within a mesenchymal hamartoma (MH) in a 15-year-old girl. Mapping of the tumor demonstrated a typical MH transforming gradually into a UES composed of anaplastic stromal cells. When evaluated by flow cytometry, the MH was diploid and the UES showed a prominent aneuploid peak. Karyotypic analysis of the UES showed structural alterations of chromosome 19, which have been implicated as a potential genetic marker of MH. The histogenesis of MH and UES is still debated, and reports of a relationship between them, although suggested on the basis of histomorphologic similarities, have never been convincing. The histologic, flow cytometric, and cytogenetic evidence reported herein suggests a link between these two hepatic tumors of the pediatric population.     

Eosinophilia and sarcoma of the small intestine (author's transl)

Eosinophilia occurs as a concomitant sign in multiple diseases of different etiology. Especially its occurrence in malignant diseases is still unknown. In three cases of primary sarcoma of the small intestine a constant eosinophilia is described. In one case this pronounced eosinophilia was present long before the final diagnose was made. The eosinophilic count was highest in the presence of clear symptoms of obstruction and normalized after surgical removal of the tumor. In one case a relapse was indicated by an increasing eosinophilic count. It is difficult to diagnose the sarcoma of the small intestine especially in its early stage because of the lack of typical symptoms. Concerning the described cases it is therefore pointed out, that in connection with undifferentiated abdominal complaints an obscure eosinophilia should be considered to be due to sarcomatous small bowel disease.