A PEER-REVIEWED JOURNAL OF RESEARCH AND CLINICAL MEDICINEISSN 1727-2378 (Print)         ISSN 2713-2994 (Online)
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Extrahepatic Portal Hypertension in Paroxysmal Nocturnal Hemoglobinuria: Clinical Case

For citation: A. V. Dezhenkova, O. F. Nikulina, N. V. Tsvetaeva, E. A. Kitsenko, E. P. Sysoeva, E. A. Lukina. Extrahepatic Portal Hypertension in Paroxysmal Nocturnal Hemoglobinuria: Clinical Case. Doctor.Ru. 2016; 5(122): 52–55.
8 April 00:00

Purpose of the Paper: 1) To outline the difficulties of the differential diagnosis between paroxysmal nocturnal hemoglobinuria (PNH) and a rare thrombotic complication — extrahepatic portal hypertension complicated by recurrent gastrointestinal hemorrhage, and 2) to describe the difficulties in determining optimal treatment approaches in such situations.

Key Points: PNH is a rare acquired clonal blood disorder. The main characteristics of classical PNH include chronic intravascular hemolysis and a high rate of thrombotic complications that lead to irreversible damage of vital organs. Nowadays, the diagnosis of PNH relies on flow cytometry to identify the abnormal population of cells deficient in superficial GPI-anchored proteins. The implementation of immunophenotyping of PNH clone cells into clinical practice has significantly improved the diagnosis of this condition.

Difficulties in the differential diagnosis of PNH are illustrated by a clinical case of portal vein thrombosis and extrahepatic portal hypertension complicated by recurrent gastrointestinal hemorrhage in a female patient.

Conclusion: When diagnosed at late stages, PNH is associated with severe thrombotic complications that damage vital organs or organ systems, which may become the main clinical problem and result in death.

A. V. Dezhenkova — Hematological Scientific Center, Moscow. E-mail: dezhenkova@list.ru

O. F. Nikulina — Hematological Scientific Center, Moscow. E-mail: nikoulinaof@mail.ru

N. V. Tsvetaeva — Hematological Scientific Center, Moscow. E-mail: tsvetaeva.n@blood.ru

E. A. Kitsenko — B. V. Petrovsky Russian Research Surgical Center, Moscow. E-mail: kitsenko-surgeon@mail.ru

E. P. Sysoeva — Hematological Scientific Center, Moscow. E-mail: sysoeva.e@blood.ru

E. A. Lukina — Hematological Scientific Center, Moscow. E-mail: lukina.e@blood.ru


Доктор.ру
8 April 00:00
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