Intestinal Infarction in Paroxysmal Nocturnal Hemoglobinuria

Blum, Stuart F.; Gardner, Frank H.

doi:10.1056/nejm196605192742008

Cited by 24 publications

(5 citation statements)

References 7 publications

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“…Our patient had no clinical or pathologic evidence of acute or chronic inflammatory bowel disease. There have been reports of intestinal ischemia because of PNH 6–9 ; but our patient had no history of acute or chronic intestinal ischemia. We can only estimate that stricture was due to excessive healing process after hematoma because there was no proven reason for stricture formation.…”

Section: Discussionmentioning

confidence: 68%

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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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Section: Discussionmentioning

confidence: 68%

“…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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“…In these cases and others [ 171, no pathologic confirmation of the abnormalities seen on radiographs was made, although it is interesting to note that the patient surviving two years ultimately succumbed from infarction and perforation of the small intestine [34]. However, in 1966 Blum and Gardner reported the successful surgical treatment of two PNH patients with intestinal infarction [24]. Our patient represents the third such case of pathologically proven intestinal infarction salvaged by aggressive surgical intervention.…”

Section: Discussionmentioning

confidence: 73%

“…As exploratory surgery in these patients is associated with morbidity and mortality [6,12], and true surgical emergencies are rare when compared with episodes of abdominal symptomatology, conservative management is generally advocated [9,20]. Many standard surgical texts and articles do not discuss PNH in the context of differential diagnosis for conditions leading to intestinal ischemia or the broader category of the acute abdomen [21-231, although acute cholecystitis [8,9], peptic ulcer disease [8,9], splenic rupture [ 171, and intestinal infarction [24] are conditions that are specific complications of PNH that demand surgical intervention. In contrast, the Budd-Chiari syndrome is a clinical situation demanding aggressive medical management [25,26] as is mesenteric venous thrombosis without infarction of the bowel and both must be differentiated from the causes of an acute abdomen.…”

Section: Discussionmentioning

confidence: 99%

Intestinal infarction caused by paroxysmal nocturnal hemoglobinuria

1984

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A patient with paroxysmal nocturnal hemoglobinuria of 14 years duration presented with severe abdominal pain and fever. On admission, his hematocrit had fallen to 19% from his usual level of approximately 30%, and stools were positive for occult blood. Dilated loops of small bowel with air fluid levels were noted on radiographs, and sonography revealed free intraperitoneal fluid. Several sections of gangrenous jejunum and ileum were resected at exploratory laparotomy, and mesenteric venous thromboses were present. This is the second report of pathologically proven intestinal infarction in a patient with paroxysmal nocturnal hemoglobinuria who has survived this abdominal emergency with surgical intervention. Although abdominal pain in patients with paroxysmal nocturnal hemoglobinuria has frequently been attributed to mesenteric venous thromboses, this has rarely been documented either during life or at autopsy.

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“…Of note, while intestinal ischemia has been postulated to be the cause of recurrent bouts of abdominal pain in patients with PNH [ 28 , 29 ], there are few data concerning a direct ante-mortem evidence of this aspect [ 30 ].…”

Section: Discussionmentioning

confidence: 99%

New Insights in Abdominal Pain in Paroxysmal Nocturnal Hemoglobinuria (PNH): A MRI Study

et al. 2015

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IntroductionAbdominal pain in PNH has never been investigated by in-vivo imaging studies. With MRI, we aimed to assess mesenteric vessels flow and small bowel wall perfusion to investigate the ischemic origin of abdominal pain.Materials and MethodsSix PNH patients with (AP) and six without (NOP) abdominal pain underwent MRI. In a blinded fashion, mean flow (MF, quantity of blood moving through a vessel within a second, in mL·s-1) and stroke volume (SV, volume of blood pumped out at each heart contraction, in mL) of Superior Mesenteric Vein (SMV) and Artery (SMA), areas under the curve at 60 (AUC60) and 90 seconds (AUC90) and Ktrans were assessed by two operators.ResultsMean total perfusion and flow parameters were lower in AP than in NOP group. AUC60: 84.81 ± 11.75 vs. 131.73 ± 18.89 (P < 0.001); AUC90: 102.33 ± 14.16 vs. 152.58 ± 22.70 (P < 0.001); Ktrans: 0.0346 min-1 ± 0.0019 vs. 0.0521 ± 0.0015 (P = 0.093 duodenum, 0.009 jejunum/ileum). SMV: MF 4.67 ml/s ± 0.85 vs. 8.32 ± 2.14 (P = 0.002); SV 3.85 ml ± 0.76 vs. 6.55 ± 1.57 (P = 0.02). SMA: MF 6.95 ± 2.61 vs. 11.2 ± 2.32 (P = 0.07); SV 6.52 ± 2.19 vs. 8.78 ± 1.63 (P = 0.07). We found a significant correlation between MF and SV of SMV and AUC60 (MF:ρ = 0.88, P < 0.001; SV: ρ = 0.644, P = 0.024), AUC90 (MF: ρ = 0.874, P < 0.001; SV:ρ = 0.774, P = 0.003) and Ktrans (MF:ρ = 0.734, P = 0.007; SV:ρ = 0.581, P = 0.047).ConclusionsPerfusion and flow MRI findings suggest that the impairment of small bowel blood supply is significantly associated with abdominal pain in PNH.

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Intestinal Infarction in Paroxysmal Nocturnal Hemoglobinuria

Cited by 24 publications

References 7 publications

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

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

Intestinal infarction caused by paroxysmal nocturnal hemoglobinuria

New Insights in Abdominal Pain in Paroxysmal Nocturnal Hemoglobinuria (PNH): A MRI Study

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