5
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      A case report of extramedullary haematopoiesis within left ventricle myocardium and apical thrombus in acute heart failure: diagnosis, treatment, and long-term outcome

      case-report

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Background 

          Extramedullary haematopoiesis (EMH) within myocardium is a rare phenomenon, and its occurrence in left ventricle myocardium or apical thrombus of a young female has never been reported.

          Case summary 

          A 23-year-old active female with progressive worsening of dyspnoea. A transthoracic echocardiogram demonstrated a left ventricular ejection fraction of 10–15% and apical thrombus. Bilateral upper extremity Doppler showed deep venous thrombus in the left arm and superficial vein thrombus in both arms. She had reduced activity of antithrombin III, deficiency of protein C and S. Computed tomography of the head showed right thalamic infarct. Having failed optimal medical therapy, rapidly worsening of symptoms (New York Heart Association Class IV and clinical Class C) and cardiogenic shock, she underwent HeartWare ® left ventricular assist device (LVAD) placement as a bridge to heart transplant. Intraoperative apical thrombus was carefully extracted while maintaining adequate anticoagulation with heparin infusion. Pathology report of the excised apical myocardium and thrombus demonstrated haematopoietic cells. Twenty-six months since LVAD implantation, she remains active and Status 7 on transplant list (due to body mass index) without any further episodes of thromboembolic events.

          Discussion

          We report an unprecedented case of an active young female with EMH within left ventricular myocardium and apical thrombus. Although redirected differentiation and embolic haematopoietic cells seem to explain this phenomenon, the exact pathophysiology remains unknown. Despite having pre-existing apical thrombus and acute deep vein thrombus, the key towards success was meticulous extraction of apical thrombus while preserving inherent trabecular architecture and adequate anticoagulation.

          Related collections

          Most cited references5

          • Record: found
          • Abstract: found
          • Article: not found

          Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment.

          To define associated clinical conditions, pathology, natural history, and treatment outcome of nonhepatosplenic extramedullary hematopoiesis (NHS-EMH). We retrospectively reviewed the medical charts of all patients identified as having NHS-EMH from 1975 to 2002. Diagnosis was made by tissue biopsy, fine-needle aspiration biopsy, or radionuclide bone marrow scanning. We identified 27 patients with antemortem diagnosis of NHS-EMH. The most common associated condition and disease site were myelofibrosis with myeloid metaplasia (MMM) (in 18 patients [67%]) and the vertebral column (in 7 patients [26%]; all involving the thoracic region), respectively. At the time of diagnosis of NHS-EMH, concurrent splenic EMH (in 22 patients [82%]; 15 [56%] had undergone splenectomy) and red blood cell transfusion dependency (in 12 patients [44%]) were prevalent. Of the 27 patients, 9 (33%) required no specific therapy. Specific therapy was radiation (in 7 patients with a 71% response rate) and surgical excision (in 6 patients with a 67% response). Treatment-associated complications were limited to surgery. Radiation therapy was not used in the non-MMM group, but low-dose radiation therapy was used in the MMM group for paraspinal or intraspinal EMH (median dose, 1 Gy; range, 1-10 Gy), pleural or pulmonary disease (median dose, 1.25 Gy; range, 1.00-1.50 Gy), and abdominal or pelvic disease (median dose, 2.02 Gy; range, 150-4.50 Gy). Median survival after the diagnosis of NHS-EMH was 13 months in the MMM group and 21 months in the non-MMM group. This retrospective study suggests that NHS-EMH is rare, is often associated with MMM, and preferentially affects the thoracic spinal region. Asymptomatic disease may require no specific treatment, whereas symptomatic disease is best managed with low-dose radiation therapy.
            Bookmark
            • Record: found
            • Abstract: not found
            • Article: not found

            Some speculations on the myeloproliferative syndromes.

            W DAMESHEK (1951)
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Myocardial extramedullary hematopoiesis: a clinicopathologic study.

              Extramedullary hematopoiesis (EMH) after fetal development is uncommon and is most often seen in patients who have hematologic disorders. EMH unassociated with hematologic disease is rare. After the recent observation of EMH in a myocardial infarct, we sought to determine the frequency and clinicopathologic setting of EMH in myocardial tissues submitted for pathologic examination. Hematoxylin and eosin (H&E)-stained sections from 805 consecutive myocardial samples (207 surgical specimens, 598 autopsy specimens) were examined retrospectively. The presence of immature erythroid or myeloid cell clusters in intramyocardial capillaries or stroma was considered sufficient for the diagnosis of EMH. Immunoperoxidase studies confirming the nature of the hematopoietic cell infiltrate were performed in selected cases. Foci of EMH (often multiple) were identified in 15 of 207 surgical hearts (7.2%) and in 22 of 598 autopsy hearts (3.7%). Patient ages (exclusive of premature infants) ranged from 2 weeks to 73 years (median, 13 years). Twenty-four of 37 (65%) EMH-positive cases were associated with infarcts in various stages of repair (accounting for 11 of 68 [16.2%] of all infarcts in surgical specimens and 13 of 86 [15.1%] of infarcts in autopsy specimens). Acute infarcts less than 72 hours old, excluding those with acute extension, were not associated with EMH. Viral myocarditis and myocardial hypertrophy with fibrosis accounted for primary diagnoses in the nonischemic, EMH-positive surgical cases, whereas seven of nine nonischemic, EMH-positive autopsy cases involved premature or term infants with no obvious myocardial disease. Another autopsy patient had sarcoidosis with myelophthisic involvement of her bone marrow and represented one of only two cases overall in which a hematopoietic disorder was coexistent or suspected. Myocardial EMH is relatively common after myocardial infarct but is rarely encountered in normal or nonischemic myocardium. Its presence in healing but not early acute stages of infarct suggests that EMH results from inflammation- or repair-associated trophic factors, not from ischemia itself.
                Bookmark

                Author and article information

                Contributors
                Role: Handling Editor
                Role: Editor
                Role: Editor
                Role: Editor
                Role: Editor
                Journal
                Eur Heart J Case Rep
                Eur Heart J Case Rep
                ehjcr
                European Heart Journal: Case Reports
                Oxford University Press
                2514-2119
                June 2019
                04 May 2019
                04 May 2019
                : 3
                : 2
                : ytz065
                Affiliations
                [1 ]Department of Medicine, University of Pittsburgh Medical Center, 200 Lothrop St, Pittsburgh, PA, USA
                [2 ]Department of Medicine, Baystate Medical Center, 759 Chestnut St, Springfield, MA, USA
                [3 ]Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, 200 Lothrop St, Pittsburgh, PA, USA
                Author notes
                Corresponding author. Tel: +1 412-999-8606, Email: 84pdeepak@ 123456gmail.com
                Author information
                http://orcid.org/0000-0002-7780-1047
                http://orcid.org/0000-0003-1421-7123
                Article
                ytz065
                10.1093/ehjcr/ytz065
                6601185
                1c5d659d-fa56-456e-a62d-b66316e7076b
                © The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 06 October 2018
                : 11 April 2019
                Page count
                Pages: 6
                Categories
                Case Reports

                extramedullary haematopoiesis,left ventricle myocardium,left ventricular assist device,prothrombotic state,apical thrombus,case report

                Comments

                Comment on this article