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Case Report
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| A case of classic paroxysmal nocturnal hemoglobinuria | ||||||
| Krishnamoorthy Seetharaman1, Suja Lakshmanan1, Ramakrishnan. S. R.2, Giridhar Muthu3 | ||||||
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1MD, Gen Med., Assistant Professor, Department of Medicine, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India.
2MD, Gen Med., Professor, Department of Medicine, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India. 3MD, Gen Med., Post Graduate, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India. | ||||||
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| How to cite this article |
| Seetharaman K, Lakshmanan S, Ramakrishnan SR, Muthu G. A case of classic paroxysmal nocturnal hemoglobinuria. Int J Case Rep Images 2015;6(6):370–375. |
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Abstract
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Introduction:
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia characterized by a triad of intravascular hemolysis, pancytopenia and tendency for venous thrombosis. Patients with PNH present with these features which occur in various combinations as described in this case report. Several episodes of intravascular hemolysis result in hemoglobinuria associated with thrombosis at unusual sites and these patients may have varying degree of bone marrow disorders. Diagnosis can be confirmed by flow cytometry of blood granulocytes and FLAER assays. Management was supportive with transfusion and treatment of thrombosis in the past. But in the recent years the evolution of treatment strategies like hemopoietic stem cell transplantation and complement inhibition with eculizumab though very costly have been shown to be very effective.
Case Report: Here we report a young girl who presented with abdominal pain, distension with a history of headache and jaundice. On evaluation, we found there was bicytopenia with evidence for hemolytic anemia and venous thrombosis of cerebral venous sinuses, hepatic veins and intrahepatic portion of IVC. With these clinical features, we suspected paroxysmal nocturnal hemoglobinuria which was later confirmed by flow cytometry. Conclusion: Having diagnosed her disease, we had to decide on various treatment options like eculizumab, hemopoietic stem cell transplantation which are efficient therapies for PNH. When these modalities are not possible in our case we had to adopt conservative management. | |
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Keywords:
Budd–Chiari syndrome, Eculizumab, Multiple venous thrombosis, Paroxysmal nocturnal hemoglobinuria (PNH)
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Author Contributions
Krishnamoorthy Seetharaman – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article and Final approval of the version to be published Suja Lakshmanan – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article and Final approval of the version to be published Ramakrishnan SR – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article and Final approval of the version to be published Giridhar Muthu – Acquisition of data, Analysis and interpretation of data, Critical revision of the article and Final approval of the version to be published |
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Guarantor of submission
The corresponding author is the guarantor of submission. |
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Source of support
None |
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Conflict of interest
Authors declare no conflict of interest. |
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Copyright
© 2015 Krishnamoorthy Seetharaman et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information. |
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About The Authors
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