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[Myelosis funicularis as a result of secondary vitamin B12 deficiency in a 9-year-old girl].

UNLABELLED: A nine-year-old girl without aggravating family history or adverse pregnancy and neonatal outcome, who underwent a resection of the end section of the small bowel as a result of atresia in the first twenty four hours of her life, was admitted to the Department on account of spastic paresis of the lower extremities. Apathy and tiredness appeared the most striking features on the physical examination of the child; likewise the pale colour of skin and a scar on the abdomen integuments--a result of the resection. Neurological examination revealed pyramidal syndrome from the lower extremities, disturbed sense of balance with intensified with closed eyes, and deep sensibility disorders. Megaloblastic anaemia was revealed on further examination, while bone marrow image showed normoblastic erythropoiesis with the presence of megaloid cells. The extremely low level of vitamin B12 in the blood serum and a regular level of folic acid were found. The vitamin B12 absorption test (Schilling test) showed considerable impairment in the process. The inflammation of the esophagus and duodenum mucosa was confirmed in a histopathological examination. Furthermore, irregular result of somatosensory induced potentials (SEP) and conductivity test revealed some characteristics of axonal--demyelinating neuropathy in the lower extremities. MRI of the head and spinal cord was correct. The diagnostics excluded methylomalonic acidosis, multiple sclerosis and other causes of ataxia in children. The overall clinical picture, that is megaloblastic anaemia, the characteristics of the myelosis funicularis and interview data--resection of the bowel as well as other examinations confirmed that the observed disorders resulted from the impairment of vitamin B12 absorption. An appropriate treatment consisted of supplementation of vitamin B12 (intramuscular) and folic acid (orally) improved the neurological condition and hematologic parameters. CONCLUSIONS: It is necessary to monitor hematologic parameters and the neurologic condition in the group of children who underwent the resection of the ileum in infancy. If vitamin B12 deficiency symptoms occur, appropriate treatment should be duly applied.

Anemia, Megaloblastic↗

[Myelosarcoma as the 1st manifestation of myelosis].

Myelosarcoma of the nodes, middle ear, rib, and spinal epidural space was observed in five cases as preceding the diagnosis of myelosis. In two cases involving epidural localization malignant lymphoma was misdiagnosed and reclassification only at the time of postmortem. Myelosarcoma is best distinguished from other types of tumour by means of the histochemical proof of naphthol-AS-D-chloracetatesterase.

Adult↗

Acute megakaryocytic myelosis preceded by myelodysplasia. Report of a case and review of the literature.

Acute megakaryocytic myelosis represents a distinct disease entity. As shown by a survey of the literature, it is a rapidly fatal disease, not preceded by a chronic myeloproliferative disorder, and mostly refractory to cytotoxic treatment. The first manifestation is pancytopenia with initial absence but quick development of hepatosplenomegaly. In contrast to "acute myelofibrosis", which is characterized by hyperplasia of all three haematopoietic lineages, a purely megakaryocytic proliferation develops together with a slight reticulinic fibrosis. Immature megakaryocytic cells may appear in the circulation, allowing classification of the disease as a variant of acute non-lymphatic leukaemia. This view is stressed by the observation of a preceding myelodysplastic phase in the case reported here.

Bone Marrow↗

Rapid detection of ringed sideroblasts in erythremic myelosis.

When normoblasts from patients with chronic erythremic myelosis were stained with the anthraquinone dye, alizarine red S, a purple perinuclear halo appeared. This halo corresponded in location to siderotic granules stained with a conventional iron stain. The rapidity of the alizarine red S stain makes it potentially useful for the detection of pathological sideroblasts in certain refractory anemias.

Bone Marrow↗

[Intensive therapy during splenectomy in patients with subleukemic myelosis].

On the basis of analysis of splenectomies in 25 patients in the terminal stage of subleukemic myelosis--myelofibrosis with the aim of prevention and treatment of complications and better endurability of splenectomy the authors have elaborated a complex of measures for intensive therapy such as conducting the operation under protection of donor thrombocytes, limited transfusion of the whole blood, early administration of antibiotics, heparin therapy and disaggregation therapy in postoperative therapy.

Adult↗

[Morphogenesis of Gaucher's cells and cells similar to them (in myelosis)].

The cytohistological resemblance between Gaucher's cells and similar cells of the bone marrow in cases of myelosis is great but their electronoptical pictures vary,. In Gaucher's cells there are membranous limited and non-limited corpuscles, composed of typical convoluted tubules, which do not contain lipids, they may be found even in the cisterns of the rough endoplasmatic reticulum and in perinuclear cisterns. They probably represent the proteinic bearer of glucosyl-ceramids, which are extracted during standard histological techniques and therefore do not form part of the histological picture. The gradual loss of the membraneceous lining around these corpuscles is probably the expression of differently advanced states of lysosomatic break down. The Gaucherlike cells of the bone marrow are apparently macrophages with many cytolysosomes, the tubular portions of which are usually not membranous limited, do not have any tendency of screwing and they differ from the previous ones in many other details.

Bone Marrow↗

Suppression of chronic lymphocytic leukaemia by megakaryocytic myelosis.

In a 77-year-old Caucasian female B-cell chronic lymphocytic leukaemia was diagnosed and classified as stage 1 according to the Rai classification. The disease remained stable and therefore no antileukaemic therapy had to be initiated. Four years after the initial diagnosis the patient developed hepatosplenomegaly, anaemia and leukopenia. Bone marrow biopsy revealed megakaryocytic myelosis supervening upon the pre-existing chronic lymphocytic leukaemia.

Aged↗

[The characteristics of infectious immunosuppression in patients with benign subleukemic myelosis].

Sixty-five patients with subleukemic myelosis (SLM) during the stage of advanced clinical manifestations were evaluated for parameters characterizing immunologic reactivity. Infectious- inflammatory processes in SLM were found out to develop in the presence of immunity deficiency manifested by the depression of T-and phagocytic link of immunity. Superimposed infection in this pathology promotes further depression of T-link of immunity as well as activation of those cellular parameters characterizing the nonspecific defence.

Aged↗

[N2O-induced acute funicular myelosis in latent vitamin B 12 deficiency].

The neurotoxicity of nitrous oxide ("laughing gas") had already been observed in 1956 when using N2O in the long-term sedation of tetanus patients. In 1967 Parbrook described leukopenic effects during long-term exposure to N2O. It was only in 1978 that further studies were conducted on myeloneuropathies and myelodepression under the influence of N2O. The basic cause is vitamin B12 deficiency and the irreversible oxidation of coenzyme B12 by N2O. Between 1986 and 1995 eight cases of acute funicular myelosis associated with latent vitamin B12 deficiency subsequent to nitrous oxide anaesthesia were reported. In our hospital, two further patients now have this disease. Two observations must be emphasised when assessing the 10 patients mentioned above: 1. There was no long-term exposure to N2O (> 6 hrs); the periods of anaesthesia were between 1.5 and 3.5 hrs. 2. Vitamin B12 deficiency was not known preoperatively, and there was no marked pernicious anaemia, so that the only pointers to the risk patients were supplied by the mean corpuscular haemoglobin (MCH) and the mean corpuscular volume (MCV) of the blood picture.

Anesthesia, Inhalation↗