Paroxysmal nocturnal hemoglobinuria as a marker for clonal myelopathy

Graham, David L.; Gastineau, Dennis A.

doi:10.1016/0002-9343(92)90201-l

Cited by 25 publications

(10 citation statements)

References 22 publications

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“…Included above are reports in the Medline literature review where the diagnosis of PNH was confirmed using the Ham test, the immune lysis test or flow cytometry. Additional patients not meeting these strict criteria may be found in Graham & Gastineau (1992) and Harris et al (1999). blasts or leukaemia, compared with two out of 23 of those with a simple karyotypic abnormality (P 0´01).…”

Section: Discussionmentioning

confidence: 99%

“…In PNH, the marrow cellularity can vary from hypocellular to hypercellular, often with erythroid hyperplasia. In two large series of PNH patients, a concurrent diagnosis of MDS was made in 4% and 11% respectively (Graham & Gastineau, 1992; Socie et al , 1996). Cytogenetically abnormal clones have been reported in up to 4% of patients with AA (Appelbaum et al , 1987).…”

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confidence: 99%

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Cytogenetic and morphological abnormalities in paroxysmal nocturnal haemoglobinuria

et al. 2001

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Paroxysmal nocturnal haemoglobinuria (PNH) is characterized by the expansion of a haematopoietic stem cell clone with a PIG‐A mutation (the PNH clone) in an environment in which normal stem cells are lost or failing: it has been hypothesized that this abnormal marrow environment provides a relative advantage to the PNH clone. In patients with PNH, generally, the karyotype of bone marrow cells has been reported to be normal, unlike in myelodysplastic syndrome (MDS), another clonal condition in which cytogenetic abnormalities are regarded as diagnostic. In a retrospective review of 46 patients with a PNH clone, we found a karyotypic abnormality in 11 (24%). Upon follow‐up, the proportion of cells with abnormal karyotype decreased significantly in seven of these 11 patients. Abnormal morphological bone marrow features reminiscent of MDS were common in PNH, regardless of the karyotype. However, none of our patients developed excess blasts or leukaemia. We conclude that in patients with PNH cytogenetically abnormal clones are not necessarily malignant and may not be predictive of evolution to leukaemia.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Cytogenetic and morphological abnormalities in paroxysmal nocturnal haemoglobinuria

et al. 2001

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“…Episodic substernal or abdominal pain is present in about 30% of patients with PNH, and may be due to thrombosis of the portal, mesenteric, or hepatic veins (Budd-Chiari syndrome) (20.21). PNH sometimes arises in a clinical setting of drug-induced marrow aplasia, myelofibrosis, niyelodysplasia, or erythroleukemia, and patients in whom the diIjease develops have increased frequency of acute myelogenous leukemia (22,23). Evans' syndrome, autoimmune hemolytic anemia, papillary endothelial hyperplasia, pulmonary hypertension, amyloidosis have also been reported in patients with PNH as well as a variety of hematologic diseases, including malignant lymphoma, myelodysplasia, and acute myelogenous leukemia (24)(25)(26)(27)(28)(29)(30)(31)(32).…”

Section: Clinical Featuresmentioning

confidence: 99%

Paroxysmal nocturnal hemoglobinuria: New discoveries about an old disease

Riley

1994

Clinical Laboratory Analysis

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“…This disorder leads to an abnormal sensitivity of the affected red blood cells and platelets to unspecific complement activation resulting in intravascular hemolysis and hypercoagulability [11]. In the bone marrow an erythroid hyperplasia has been described [5,8,15].…”

Section: Introductionmentioning

confidence: 98%

“…As frequent complications infection [24], aplastic anemia [20] or agranulocytosis [4] and mainly venous thrombembolisms [5,24] have been described. Myelodysplastic JON 1871 ■ Abstract Background Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder, leading to a deficient biosynthesis of surface proteins in hematopoetic cells.…”

Section: Introductionmentioning

confidence: 99%