Associazione Nazionale Medici Cardiologi Ospedalieri



When the hematologist has the solution – a rare case of heart failure

Biole’ Carloalberto Orbassano (Torino) – Aou San Luigi Gonzaga | Bianco Matteo Orbassano (Torino) – Aou San Luigi Gonzaga | Ulisciani Stefano Orbassano (Torino) – Aou San Luigi Gonzaga | Nangeroni Giulia Orbassano (Torino) – Aou San Luigi Gonzaga | Chinaglia Alessandra Orbassano (Torino) – Cardiologia

Introduction: cardiologists are confident with heart failure due to cardiac etiology, but sometimes this may be due to other organs' dysfunction (e.g. endocrine problems); in this case we report a very rare cause of anasarca due to hematologic disease.

Case report: A 33 year old man with a known complete left bundle branch block and without other pathologies is admitted to the ED for sickness and abdominal pain. Low platelet count, splenomegaly and an anterior mediastinal mass (53x54mm) are found. A Positron Emission Tomography (PET) reveals multiple lymphnodes captation above and under the diaphragm and at mediastinal level. Bone marrow biopsy is consistent with myelofibrosis. During the hospitalization the patient experiences an important weight gain (14 kg) with edema and worsening renal function suspected for heart failure; echocardiogram shows a globally hypokinetic left ventricle with moderately reduced ejection fraction (EF 37%), mild pericardial effusion, no relevant valve anomalies; cardiac MRI confirmed moderately depressed EF with global hypokinesia and no LGE anomalies of the myocardial wall; a clinical benefit is noted with high dose i.v. diuretics, and echocardiogram findings rapidly improve with therapy, EF 49% after decongestion; a discrepancy is noted between the severity of anasarca and echocardiographic data; ultimately, lymph node biopsy is performed, which is consistent with Castleman disease, HHV 8 negative. TAFRO (Thrombocytopenia, Anasarca, Fever, Reticulin myelofibrosis/Renal dysfunction, Organomegaly) syndrome is therefore diagnosed. Therapy with siltuximab (anti IL-6) is started with good clinical response on lymph nodes enlargement and on anasarca, and the patient is currently followed-up by the hematologists of our institution. 

Discussion: TAFRO syndrome is a very rare hematologic disease that can manifest with severe anasarca; usually it presents with non-cardiac features, but cardiologists should be aware of this disease as they could be involved in the management of this condition, especially in the fist stages when the diagnosis has not yet been made.