Intestinal infarction caused by paroxysmal nocturnal hemoglobinuria

Doukas, Michael; DiLorenzo, Paul; Mohler, Daniel N.

doi:10.1002/ajh.2830160110

Cited by 21 publications

(8 citation statements)

References 21 publications

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“…This accords well with the observation that these organisms are rarely seen in acute urinary infections, being more commonly implicated in chronic conditions, especially after use of indwelling urinary catheters. 5 The results of this study also suggest that the use of cysteine supplemented agar media has no particular advantage over MacConkey agar for patients from a general practice population presenting with acute urinary tract infections. report the results of our study in which we compared activated partial thromboplastin times (APTTs) and British ratios in 50 patients receiving long term warfarin treatment.…”

Section: Incidence Of Cysteine Dependent Escherichia Cofi In a Generamentioning

confidence: 68%

Microvascular thrombosis of the bowel in paroxysmal nocturnal haemoglobinuria.

Williamson¹,

Johnstone²,

Preston³

1987

J Clin Pathol

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Section: Incidence Of Cysteine Dependent Escherichia Cofi In a Generamentioning

confidence: 68%

Microvascular thrombosis of the bowel in paroxysmal nocturnal haemoglobinuria.

Williamson¹,

Johnstone²,

Preston³

1987

J Clin Pathol

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“…Recurrent attacks of abdominal pain are common. The possibility that a thrombosis in the portal or mesenteric veins is the cause of the pain must always be taken in consideration (14). Severe abdominal pain dominated the clinical feature of one of our patients.…”

Section: Pnh and Thrombosismentioning

confidence: 86%

Paroxysmal Nocturnal Hemoglobinuria (Membrane Defect, Pathogenesis, Aplastic Anemia, Diagnosis)

Chrobák

2000

Acta Med. (Hradec Kralove, Czech Repub.)

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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder in which intravascular hemolysis results from the somatic mutation of the totipotent stem cells causing an intrinsic defect in red cell membrane. PNH cells lack glycosylphosphatidylinositol (GPI) anchored membrane proteins. Of these proteins absence of CD 59 (MIRL - membrane inhibitor of reactive lysis, protectin) and CD 55 (DAF - decay accelerating factor) makes the PNH cells abnormally sensitive to the lytic action of complement. The defect appears to be in the somatic mutation of the X-linked PIG-A (phosphatidylinositolglycan A class) gene which participate in an early step of GPI - anchor synthesis. PNH is characterized by recurrent life threatening venous thromboses and an intimate association with aplastic anemia (AA). It seems that PNH always coexists with bone marrow failure (BMF) (37). The possible explanation may be that some GPI-anchored proteins may be a critical target recognized by immune effector cells. PNH clones not possessing these critical GPI - anchored proteins will survive because they are selectively resistant to the autoimmune assault that eliminates most normal clones. The flow cytometry of erythrocytes using anti-CD 59 and anti-CD 59 and anti-CD 55 of granulocytes has been now introduced as a very sensitive and quantitative method of PNH diagnosis able to detect PNH cells even in normal individuals (1,54). Thus it seems now clear that we must make distinction between the detection of very occasional PNH cells in patients with BMF and PNH as a clinicohematological entity. Unfortunately, we do not know the minimal content of PNH cells required to produce clinical signs of PNH (38).

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“…Since Crosby 5 reported that 54% of patients were found to have hepatic or portal thrombi, there have been four reported cases of intestinal ischemia treated successfully with surgical intervention. 6–10 …”

Section: Discussionmentioning

confidence: 99%

Delayed duodenal obstruction after intramural hematoma in a patient with paroxysmal nocturnal hemoglobinuria: A case report

Tezcaner

Ekici

Kırnap

et al. 2014

International Journal of Surgery Case Reports

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INTRODUCTIONParoxysmal nocturnal hemoglobinuria (PNH) is a clonal stem cell disorder of hematopoietic cells. Gastrointestinal complications of PNH are rare and mostly related with intravascular thrombosis or intramural hematoma.PRESENTATION OF CASEWe describe a case of a man with PNH complicated by intramural duodenal hematoma initially treated with supportive care. Three months after his first admission; he was admitted to the emergency department with abdominal pain, nausea and vomiting. He had undergone to surgery because of duodenal obstruction was treated with duodenojejunal by-pass surgery.DISCUSSIONPatients were healed from gastrointestinal complications could suffer from gastrointestinal strictures, which cause wide spread symptoms ranging from chronic abdominal pain and anorexia to intestinal obstruction.CONCLUSIONWe report a rare intestinal obstruction case caused by stricture at the level of ligamentum Treitz with PNH. The possibility simply has to be borne in mind that strictures can be occurring at hematoma, ischemia or inflammation site of gastrointestinal tract.

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Intestinal infarction caused by paroxysmal nocturnal hemoglobinuria

Cited by 21 publications

References 21 publications

Microvascular thrombosis of the bowel in paroxysmal nocturnal haemoglobinuria.

Microvascular thrombosis of the bowel in paroxysmal nocturnal haemoglobinuria.

Paroxysmal Nocturnal Hemoglobinuria (Membrane Defect, Pathogenesis, Aplastic Anemia, Diagnosis)

Delayed duodenal obstruction after intramural hematoma in a patient with paroxysmal nocturnal hemoglobinuria: A case report

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