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Biomedical subjects

R Jiji

Publications and source records attributed to R Jiji.

10 recordsLinked to original sources

Chromosome abnormalities in acquired idiopathic sideroblastic anemia with subsequent leukemic transformation.

Chromosomal abnormalities were demonstrated in the bone marrow cultures of two patients with acquired idiopathic sideroblastic anemia (AISA). Both patients subsequently experienced leukemic transformation and developed acute myelomonocytic leukemia (type M4). A review of the literature revealed that approximately 40% of the AISA cases manifest chromosomal abnormalities, of which 20.5% underwent leukemic conversion.

Age Factors↗

Deferoxamine (Desferal)-induced toxic retinal pigmentary degeneration and presumed optic neuropathy.

Eight patients (16 eyes) developed ocular toxicity while undergoing intravenous deferoxamine mesylate (Desferal) chelation therapy for transfusional hemosiderosis. Presenting symptoms included decreased visual acuity, color vision abnormalities, and night blindness. Six patients presented as presumed retrobulbar optic neuropathy demonstrating central scotomas and color vision abnormalities. The remaining two patients presented with pigmentary changes confined either to the macula or equator. Following cessation of therapy, vision improved in all but four eyes, which did not attain their pretreatment visual acuity. Optic neuropathy resolved in all cases. However, follow-up revealed development of retinal pigmentary degeneration in seven patients, involving the macula in six and the equatorial retina in one. Fluorescein angiography and electrophysiological tests suggested toxicity at the level of retinal pigment epithelium and photoreceptors.

Aged↗

Microcytic hypochromic anemia associated with renal cell carcinoma.

Anemia, frequently hypochromic and microcytic, occurs in approximately one third of patients with renal cell carcinoma. We have described a patient with classic iron deficiency anemia and renal cell carcinoma whose blood picture reverted to normal after removal of the tumor. Abundant hemosiderin in the tumor cells is evidence that tumor cells removed iron from the circulation, causing the iron deficiency anemia.

Adenocarcinoma↗

Translocation (6;9)(p23;q34) in acute nonlymphocytic leukemia.

A consistent chromosomal abnormality t(6;9)(p23;q34) was demonstrated in the bone marrow or unstimulated peripheral blood cultures of two patients with acute nonlymphocytic leukemia (ANLL). Only two additional cases with ANLL and a similar cytogenetic finding have been reported, indicating that this translocation may represent another chromosomal abnormality specifically associated with ANLL.

Adult↗

Lysis of paroxysmal nocturnal hemoglobinuria erythrocytes by acid-activated serum.

Erythrocytes from paroxysmal nocturnal hemoglobinuria patients (PNH-E) are much more susceptible to lysis by acid-activated human serum than normal human erythrocytes. Acidification of normal human serum to pH 6.4 in the absence of erythrocytes generates this lytic activity independently of the alternative pathway of complement activation. A shift of pH of a mixture of purified human C5 and C6 to 6.4 at 0 degrees C generates a similar activity C(56)a that lyses PNH-E together with C7-C9 much more efficiently than normal erythrocytes. Since acid-activation of normal human serum occurs in the absence of C3, the acid-activated C56 appears to be the lytic principle in acidified human serum.

Animals↗

An anti-i biphasic hemolysin in chronic paroxysmal cold hemoglobinuria.

Paroxysmal cold hemoglobinuria (PCH) was diagnosed in an elderly patient with a history of chronic lymphocytic leukemia. In this case, the Donath-Landsteiner antibody demonstrated anti-i specificity rather than the more commonly reported anti-P specificity. This unusual case of a biphasic hemolysin with anti-i specificity emphasizes the importance of performing a Donath-Landsteiner test when presented with serological or clinical findings that are suggestive of PCH.

Aged↗