Clinico-Haematological Features of Paroxysmal Nocturnal Haemoglobinuria

J Coll Physicians Surg Pak. 2017 Jan;27(1):53-54.

Abstract

The aim of this study was to determine the frequency of various clinico-haematological features in patients suffering from paroxysmal nocturnal haemoglobinuria (PNH). It was an observational study carried out from October 2008 - January 2016. All the patients of PNH, diagnosed on the basis of clinical and laboratory findings and confirmed by CD55 and CD59 deficiency on red cells by means of flow cytometry, were included in the study. A total of 22 patients were diagnosed which included 18 (81.8%) males and 4 (18.1%) females. Median age was 27 years. Pallor, fever, fatigability and haemoglobinuria were the most common clinical features. Pancytopenia was seen in 13 (59.09%) and hypocellular marrow was found in 14 (63.6%) patients. One patient presented with Budd Chiari syndrome.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Age Distribution
  • Anemia, Hemolytic / diagnosis*
  • Anemia, Hemolytic / epidemiology
  • Bone Marrow / metabolism
  • Bone Marrow / pathology*
  • Cohort Studies
  • Erythrocytes / cytology
  • Female
  • Flow Cytometry / methods
  • Hemoglobinuria / diagnosis*
  • Hemoglobinuria / epidemiology
  • Hemoglobinuria, Paroxysmal / diagnosis*
  • Hemoglobinuria, Paroxysmal / epidemiology*
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Pakistan
  • Prognosis
  • Rare Diseases
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Sex Distribution

Supplementary concepts

  • CD59 Deficiency