You are here

  • Home
  • Archive
  • Volume 17, Issue 3
  • Classic paroxysmal nocturnal haemoglobinuria presenting with intestinal malabsorption syndrome, acute abdomen and acute kidney injury

Article Text

Article menu
Download PDFPDF
Haematology
Classic paroxysmal nocturnal haemoglobinuria presenting with intestinal malabsorption syndrome, acute abdomen and acute kidney injury
  1. Rajdeep Porel1,
  2. Anjani Kumar1,
  3. Surabhi Ajit2,
  4. Vishnu Shankar Ojha1 and
  5. Ratnadeep Biswas1
  1. 1Department of General Medicine, All India Institute of Medical Sciences - Patna, Patna, India
  2. 2Department of Pathology and Lab Medicine, All India Institute of Medical Sciences - Patna, Patna, India
  1. Correspondence to Dr Ratnadeep Biswas; ratnadeepbis2404{at}gmail.com

Abstract

A male patient in his 30s presented with complaints of acute abdominal pain, black stools and red-coloured urine. CT revealed thrombi in the splenic and left renal veins, leading to infarctions. An endoscopy displayed scalloping of the duodenal folds, indicative of intestinal malabsorption syndrome (IMS). Histopathological examination confirmed IMS. Due to the presence of intravascular haemolysis, haemoglobinuria and thrombotic complications, paroxysmal nocturnal haemoglobinuria (PNH) was suspected and subsequently confirmed by flow cytometry. Thus, a diagnosis of classic PNH with IMS and thrombotic complications was established. This unique case highlights the coexistence of PNH and IMS, resembling the complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy disease, suggesting potential shared pathophysiology.

  • Haematology (incl blood transfusion)
  • Gastroenterology
  • Malabsorption
  • Thrombosis
  • Pathology

https://doi.org/10.1136/bcr-2023-258785

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text

    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: All authors were involved with the patient care. RP and SA procured the images. RB wrote the initial draft of the manuscript. RP, AK and SA edited the manuscript. The following authors gave final approval of the manuscript: RP, AK, SA, VSO and RB.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.