Tuberc Respir Dis.  2009 Sep;67(3):244-248.

A Case of Extramedullary Hematopoiesis Presenting as a Lung Mass in a Patient with Primary Myelofibrosis

Affiliations
  • 1Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences, Seoul, Korea. cheol@kcch.re.kr
  • 2Department of Laboratory Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences, Seoul, Korea.

Abstract

Primary myelofibrosis is characterized by replacement of bone marrow with fibrotic tissue and the development of extramedullary hematopoiesis. Extramedullary hematopoiesis primarily involves the spleen and liver, but can also occur in the lungs. We report the case of an 80-year-old male who was admitted for evaluation of a lung mass and persistent thrombocytopenia. A percutaneous needle aspiration from the mass in the right lower lung showed myelopoietic cells with fatty tissue. A bone marrow biopsy revealed a hypercellular marrow with an increased number of atypical megakaryocytes. The final diagnosis was a prefibrotic stage of primary myelofibrosis leading to extramedullary hematopoiesis in the lung.

Keyword

Extramedullary hematopoiesis; Primary myelofibrosis; Lung mass

MeSH Terms

Adipose Tissue
Aged, 80 and over
Biopsy
Bone Marrow
Hematopoiesis, Extramedullary
Humans
Liver
Lung
Male
Megakaryocytes
Needles
Primary Myelofibrosis
Spleen
Thrombocytopenia

Figure

  • Figure 1 Chest X-ray findings. An airspace opacity was found in the right lower lung.

  • Figure 2 Chest CT findings. (A) Ground glass opacities and a 3 cm mass were found in the right lower lung. (B) Mild splenomegaly was noted with multiple low attenuated lesion, which are likely foci of extramedullary hematopoiesis.

  • Figure 3 Pathologic findings of percutaneous needle aspiration from the mass in the right lower lung. The myelopoietic cells with fatty tissue were suggestive of extramedullary hematopoiesis (H&E stain, ×400).

  • Figure 4 Peripheral blood smear findings. The platelet number was markedly decreased (Wright-Giemsa stain, ×1,000).

  • Figure 5 Bone marrow biopsy findings. The marrow revealed hypercellularity (>90%) with an increased number of dysplastic megakaryocytes, including megakaryocytes with hypolobulated nuclei (Wright-Giemsa stain, ×400).


Reference

1. Bowling MR, Cauthen CG, Perry CD, Patel NP, Bergman S, Link KM, et al. Pulmonary extramedullary hematopoiesis. J Thorac Imaging. 2008. 23:138–141.
2. Koch CA, Li CY, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003. 78:1223–1233.
3. Lund RE, Aldridge NH. Computed tomography of intracranial extramedullary hematopoiesis. J Comput Assist Tomogr. 1984. 8:788–790.
4. MacKinnon S, McNicol AM, Lee FD, McDonald GA. Myelofibrosis complicated by intestinal extramedullary haemopoiesis and acute small bowel obstruction. J Clin Pathol. 1986. 39:677–679.
5. Oh HA, Kang HJ, Lee G, Choi JH, Lee JL, Lee KH, et al. A case of chronic myelogenous leukemia with extramedullary hematopoiesis presenting as liver mass. Korean J Hematol. 2002. 37:80–83.
6. Lee HJ, Hong JH, Kang YH, Seo KS, Seong EY, Yu YI, et al. A case of cutaneous extramedullary hematopoiesis in idiopathic myelofibrosis. Korean J Hematol. 1997. 32:476–480.
7. Goh DH, Lee SH, Cho DC, Park SH, Hwang JH, Sung JK. Chronic idiopathic myelofibrosis presenting as cauda equina compression due to extramedullary hematopoiesis: a case report. J Korean Med Sci. 2007. 22:1090–1093.
8. Kwak HS, Lee JM. CT findings of extramedullary hematopoiesis in the thorax, liver and kidneys, in a patient with idiopathic myelofibrosis. J Korean Med Sci. 2000. 15:460–462.
9. Spivak JL. Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J, editors. Chapter 103. Polycythemia vera and other myeloproliferative diseases. Harrison's principles of internal medicine. 2008. 17th ed. New York: McGraw-Hill Co, Inc.;674–675.
10. Ward HP, Block MH. The natural history of agnogenic myeloid metaplasia (AMM) and a critical evaluation of its relationship with the myeloproliferative syndrome. Medicine (Baltimore). 1971. 50:357–420.
11. Bradford CR, Smith SR, Wallis JP. Pericardial extramedullary hematopoiesis in chronic myelomonocytic leukemia. J Clin Pathol. 1993. 46:674–675.
12. Fielding JR, Owens M, Naimark A. Intrathoracic extramedullary hematopoiesis secondary to B12 and folate deficiency: CT appearance. J Comput Assist Tomogr. 1991. 15:308–310.
13. Asakura S, Colby TV. Agnogenic myeloid metaplasia with extramedullary hematopoiesis and fibrosis in the lung: report of two cases. Chest. 1994. 105:1866–1868.
14. Kopecky KK, Moriarty AT, Antony AC, Baker MK. Extramedullary hematopoiesis in acute lymphocytic leukemia masquerading as hepatic, renal, and splenic microabscesses. AJR Am J Roentgenol. 1986. 147:846–847.
15. Kupferschmid JP, Shahian DM, Villanueva AG. Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest. 1993. 103:974–975.
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