Autoimmune Hemolytic Anemia Causing Group 5 Pulmonary Hypertension: A Rare Case

Muchammad Dzikrul Haq Karimullah, Hiradipta Ardining


Pulmonary hypertension (PH)has been associated with hemolytic anemia. The prevalence of PH in hemolytic anemia is estimated to be as high as 10-40%, and reportsarepresenting poor prognosis in this subset of patients. PH associated with autoimmune hemolytic anemia (AIHA) is still rarely discussed,and there is paucity of literature regarding its precise pathophysiology and treatment. Here, we describe a case of PH associated with AIHA. A 34-year old woman came to our center with chief complaint of dyspnea on exertion. She was previously diagnosed with AIHA with positive direct Coomb's test. Physical examination, chest X-ray and echocardiography were consistent with pulmonary hypertension. The diagnosis of group 5 pulmonary hypertension was made. Although rare, the association between chronic hemolytic anemia and PH is evident, through several mechanisms involving nitric oxide inactivation, direct injury oftheendothelium, oxidative damage, thromboembolic formation, and left ventricular dysfunction. The management of PH in hemolytic disorders comprises treatment of underlying hemolytic disorder and PH-specific therapies. For PH specific therapy, to date, there are no therapies that have been fully studied for these specific patient population. Our patient was given bisoprolol, furosemide, amlodipine, spironolactone, candesartan, beraprost sodium and sildenafil. On follow up twomonths later, her functional status was improved. In summary, PH associated with AIHA develop via multifactorial and complex mechanisms. PH in AIHA could be detected with meticulous history taking, physical examination, chest X-ray and echocardiography, and treatment with vasodilating agents were shown to improve the PH.


pulmonary hypertension, autoimmune hemolytic anemia, pulmonary arterial hypertension

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Orem C. Epidemiology of pulmonary hypertension in the elderly. J Geratr Cardiol [Internet]. 2017; 14(1):11-16. doi:

Hoeper MM, Gibbs SR. The changing landscape of pulmonary arterial hypertension and implications for patient care. European Respiratory Review [Internet]. 2014; 23:450-7. doi: 10.1183/09059180.00007814

Hoeper MM, Kramer T, Pan Z, Eichstaedt CA, Spiesshoefer J, Benjamin N, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. European Respiratory Journal [Internet]. 2017;50. doi: 10.1183/13993003.00740-2017

Wirejatne DT, Lajkosz K, Brogly SB, Lougheed D, Jiang L, Housin A, et al. Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension. Circulation: Cardiovascular Quality and Outcomes [Internet]. 2018; 11:e003973. doi:

Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Canada: Elsevier; 2019. 4316 p

Opotowsky AR. Clinical Evaluation and Management of Pulmonary Hypertension in the Adult with Congenital Heart Disease. Circulation [Internet]. 2015;131:200-210. doi:

Augustine DX, Bradshaw LDC, Willis J, Harkness A, Ring L, Grapsa J, et al. Echocardiographic assessment of pulmonary hypertension: a guideline protocol from the British Society of Echocardiography. Echo Res Pract [Internet]. 2018; 5(3): G11-G24. doi: 10.1530/ERP-17-0071

Hoffman R, Benz EJ, Shattil SJ, Furie B, Silberstein LE, McGlave P, et al. Hematology Basic Principles and Practice. 5th ed. Churchill Livingstone; 2009. 645 p

Wahl S, Vichinsky E. Pulmonary hypertension in hemolytic anemias. F1000 Med Rep [Internet]. 2010; 2:10. doi:

Zhang Y, Qui Y, Zhu J, Gao D. Pulmonary hypertension associated with autoimmune hemolytic anemia: A case report. International Journal of Cardiology [Internet]. 2007; 115: e1-e2.

Singh S, Sharma S. High-Output Cardiac Failure [Internet]. Treasure Island (FL): StatPearls Publishing; 2020. Available from:

Haw A, Palevsky HI. Pulmonary Hypertension in Chronic Hemolytic Anemias: Pathophysiology and Treatment. Respiratory Medicine [Internet]. 2018; 137: 191-200. doi:

Prins KW, Thenappan T, Weir EK, Kaira R, Pritzker M, Archer SL. Repurposing Medications for Treatment of Pulmonary Arterial Hypertension: What's Old is New Again. Journal of the American Heart Association. 2019 [cited 2020 May]. 2019;8:e011343. Available from:



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