Pneumologie (Stuttgart, Germany) · 2022
[Pulmonary Hypertension and Polycythemia vera].
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Westhoff Michael, Litterst Patric, Kreppein Ursula
Аннотация
INTRODUCTION: Chronic myeloproliferative diseases are rare causes of PH class 5 according to Nice classification 2018. The present case reports show different courses, on the one hand with a primary manifestation of a PH and subsequently a PV, on the other hand with the development of a PH in the context of a PV.
CASE REPORTS: 1) At first contact, a 75-year-old female patient who complained progressive dyspnea and had evidence of stress-PH in the right heart catheter. During the course she developed a resting PH of up to 70 mmHg systolic despite initial monotherapy and subsequent dual therapy for PH. After 5 years she had the diagnosis of polycythemia vera, treated with hydroxycarbamide and subsequent phlebotomies. In the further course increasing cardiac decompensation and death. 2) 74-year-old female patient at the time of diagnosis of chronic megakaryocytic-granulocytic myelosis. After 7 years, evidence of polycythemia vera (V617F mutation in the JAK2 gene), a monoclonal gammopathy. In the case of splenomegaly, irradiation of the spleen was carried out and, after 1 year, therapy with ruxolitinib was started. After another 2 years, with increasing dyspnea, pulmonary hypertension (CTEPH) with a PA-mean of 43 mmHg and a PVR of 4.5 WE were detected. With anticoagulation and riociguat therapy exercise capacity and PA pressures were only temporarily improved. Within 1 year restrictive ventilation, hypoxemia, heart failure (EF 45 %) with leading right heart decompensation and cardiorenal syndrome developed. Dialysis showed only short-term recompensation, and the patient died.
DISCUSSION: The case reports are characterized by a combination of PV and PH, with different temporal sequence, as well as only a low influence of PH-specific therapy, with subsequent progressive cardiac decompensation. Thus, they reflect the different etiologies, clinical manifestations, and the low therapeutic influence of PH in myeloproliferative disorders. The value of PH-specific therapy remains unclear, especially in view of different pathomechanisms in the genesis of PH.
CONCLUSION: Patients with myeloproliferative diseases require screening for PH. In the course of PH, myeloproliferative disease can unmask or develop. The therapeutic influence on PH is limited.
CASE REPORTS: 1) At first contact, a 75-year-old female patient who complained progressive dyspnea and had evidence of stress-PH in the right heart catheter. During the course she developed a resting PH of up to 70 mmHg systolic despite initial monotherapy and subsequent dual therapy for PH. After 5 years she had the diagnosis of polycythemia vera, treated with hydroxycarbamide and subsequent phlebotomies. In the further course increasing cardiac decompensation and death. 2) 74-year-old female patient at the time of diagnosis of chronic megakaryocytic-granulocytic myelosis. After 7 years, evidence of polycythemia vera (V617F mutation in the JAK2 gene), a monoclonal gammopathy. In the case of splenomegaly, irradiation of the spleen was carried out and, after 1 year, therapy with ruxolitinib was started. After another 2 years, with increasing dyspnea, pulmonary hypertension (CTEPH) with a PA-mean of 43 mmHg and a PVR of 4.5 WE were detected. With anticoagulation and riociguat therapy exercise capacity and PA pressures were only temporarily improved. Within 1 year restrictive ventilation, hypoxemia, heart failure (EF 45 %) with leading right heart decompensation and cardiorenal syndrome developed. Dialysis showed only short-term recompensation, and the patient died.
DISCUSSION: The case reports are characterized by a combination of PV and PH, with different temporal sequence, as well as only a low influence of PH-specific therapy, with subsequent progressive cardiac decompensation. Thus, they reflect the different etiologies, clinical manifestations, and the low therapeutic influence of PH in myeloproliferative disorders. The value of PH-specific therapy remains unclear, especially in view of different pathomechanisms in the genesis of PH.
CONCLUSION: Patients with myeloproliferative diseases require screening for PH. In the course of PH, myeloproliferative disease can unmask or develop. The therapeutic influence on PH is limited.