Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sideroblastic anemia is an extremely rare disorder in children. This report describes a 9-year 4-month-old girl with severe refractory anemia with ringed sideroblasts (RARS) that progressed to severe bone marrow aplasia. Ultrastructural studies revealed the presence of abundant intramitochondrial deposits of iron in erythroblasts similar to that observed in adults with this disorder. Although acid ferrocyanide staining confirmed the ferric valence of the iron deposits, they lacked morphologic and cytochemical characteristics associated with ferritin and hemosiderin. Bone marrow culture showed decreased or absent CFU-GEMM, CFU-GM, CFU-E, and BFU-E. Erythrocyte uroporphyrinogen I synthase, aminolevulinic acid dehydratase, uroporphyrinogen decarboxylase, urinary porphyrins, porphobilinogen, and aminolevulinic acid were normal. Free red cell protoporphyrin was increased. Therapy with corticosteroid and androgens was totally ineffective. The aplastic bone marrow in this child appeared to represent the end stage of RARS and differed from adults with RARS, who more frequently demonstrate a chronic course, often with the onset of leukemia as a terminal sequela. Although this case documents the occurrence of RARS in a child, additional reports of children with this disorder will be required to determine the prognosis and natural history of RARS in children.
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PMID:Severe refractory anemia with ringed sideroblasts and bone marrow aplasia in a child. 155 Feb 66

A case of a familial porphyria cutanea tarda (PCT-II) is reported in which the clinically overt form of PCT was provoked by factors relating to chronic lymphoid leukemia (CLL). Typical lesions of PCT developed on a 55-year-old woman after several blood transfusions and chlorambucil treatment. Besides these provoking factors, cytomegalovirus (CMV) infection was diagnosed. Erythrocyte uroporphyrinogen decarboxylase activity was about 50% of normal in the patient and in her two children. This case supports the suggestion that development of PCT in patients with hematological disorders is more than coincidental but may in fact be provoked by exogenous factors relating to the treatment of leukemia.
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PMID:Porphyria cutanea tarda and chronic lymphoid leukemia. 901 95