|Year : 2017 | Volume
| Issue : 2 | Page : 143-144
F-18-Fluorodeoxyglucose positron emission tomography/computed tomography appearance of extramedullary hematopoesis in a case of primary myelofibrosis
Anirban Mukherjee1, Chandrasekhar Bal1, Madhavi Tripathi1, Chandan Jyoti Das2, Shamim Ahmed Shamim1
1 Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||16-Mar-2017|
Dr. Shamim Ahmed Shamim
Department of Nuclear Medicine All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A 44-year-old female with known primary myelofibrosis presented with shortness of breath. High Resolution Computed Tomography thorax revealed large heterogeneously enhancing extraparenchymal soft tissue density mass involving bilateral lung fields. F-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography revealed mildly FDG avid soft tissue density mass with specks of calcification involving bilateral lung fields, liver, and spleen. Subsequent histopathologic evaluation from the right lung mass was suggestive of extramedullary hematopoesis.
Keywords: Extramedullary hematopoesis, F-18-fluorodeoxyglucose positron emission tomography/computed tomography, primary myelofibrosis
|How to cite this article:|
Mukherjee A, Bal C, Tripathi M, Das CJ, Shamim SA. F-18-Fluorodeoxyglucose positron emission tomography/computed tomography appearance of extramedullary hematopoesis in a case of primary myelofibrosis. Indian J Nucl Med 2017;32:143-4
|How to cite this URL:|
Mukherjee A, Bal C, Tripathi M, Das CJ, Shamim SA. F-18-Fluorodeoxyglucose positron emission tomography/computed tomography appearance of extramedullary hematopoesis in a case of primary myelofibrosis. Indian J Nucl Med [serial online] 2017 [cited 2019 Dec 12];32:143-4. Available from: http://www.ijnm.in/text.asp?2017/32/2/143/202235
A 44-year-old female presented with recurrent episodes of epistaxis. On subsequent evaluation, she was found to have leukoerythrobastic picture in peripheral blood with bicytopenia. On physical examination, massive splenomegaly was noted. A provisional diagnosis of primary myelofibrosis was made based on the above-mentioned findings. For further confirmation, bone marrow biopsy was performed which revealed a hypercellular marrow (60%), polymorphous trilineage hematopoiesis and moderate to severe reticulin fibrosis. So based on bone marrow biopsy, primary myelofibrosis was confirmed. The patient was initiated with lenalidomide and steroid. She was improved on the same with subsequent improvement in the platelet count and became transfusion independent. After 1 year of disease-free interval, she presented with shortness of breath. HRCT thorax revealed large heterogeneously enhancing extraparenchymal soft tissue density mass involving bilateral lung fields. For further evaluation of the lung mass fluorodeoxyglucose (FDG) positron emission tomography/computed tomography was performed which revealed minimally FDG avid soft tissue density mass with dense calcification involving liver and spleen [Figure 1]A-C. Along with that large heterogeneously enhancing extraparenchymal soft tissue density mass noted with specks of calcification along costal pleura projecting into bilateral lung parenchyma showing minimal FDG uptake in the periphery with non FDG avid areas in the centre possibly represents area of necrosis [Figure 1]D-I. Based on her initial diagnosis of primary myelofibrois and low FDG avidity of the lesions a provisional diagnosis of extramedullary hematopoesis (EMH) was made and histopathologic confirmation was suggested. Subsequent histopathologic evaluation from the right lung mass confirmed the diagnosis of EMH.
|Figure 1: (a-c) FDG PET-CT was performed which revealed minimally FDG avid soft tissue density mass with dense calcification involving liver and spleen [. (d-i) Along with that large heterogeneously enhancing extraparenchymal soft tissue density mass noted with specks of calcification along costal pleura projecting into bilateral lung parenchyma showing minimal FDG uptake in the periphery with non FDG avid areas in the centre possibly represents area of necrosis|
Click here to view
Primary myelofibrois is a BCR-ABL negative myeloproliferative neoplasm, characterized by progressive bone marrow fibrosis and ineffective erythropoiesis. EMH is a well-recognized phenomenon of this disease process. The pathophysiology of EMH is thought to be associated with the constitutive mobilization of CD34+ cells into the peripheral blood. This dysregulation of hematopoietic stem cell trafficking likely ultimately leads to the seeding of extramedullary sites. Liver and spleen are the most common sites of EMH, but it has been reported in skin, breast, gastrointestinal tract, lymph node, lung, thyroid gland, and conjunctiva., To date, only few cases have reported FDG PET-CT appearance of EMH,, with EMH detected as a benign mass with low SUVmax values and normal appearance of the tissue, which is consistent with the findings of our case. The concurrent presence of an underlying hematopoietic disorder may further suggest a diagnosis of EMH. Thus in patients with hematologic disorder, EMH should be kept as a differential diagnosis in evaluation of any sift tissue mass and FDG PET-CT may be helpful in this case by distinguishing benign EMH which demonstrate low SUVmax from malignant lesions with high SUVmax values.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, et al.
“Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT),”. Leukemia Res 2007;31:737-40.
Gregory SA, Mesa RA, Hoffman R, Shammo JM. “Clinical and laboratory features of myelofibrosis and limitations of current therapies.”. Clin Advances Hematolo Oncolo 2011;9:1-16.
Georgiades CS, Neyman EG, Francis IR, Sneider MB, Fishman EK. “Typical and atypical presentations of extramedullary hemopoiesis.”. Am J Roentgenol 2002;179:1239-43.
Leibundgut EO, Horn MP, Brunold C, Pfanner-Meyer B, Marti D, Hirsiger H, et al.
“Hematopoietic and endothelial progenitor cell trafficking in patients with myeloproliferative diseases.” Haematologica 2006;91:1465-72.
Remstein ED, Kurtin PJ, Nascimento AG. Sclerosing extramedullary hematopoietic tumor in chronic myeloproliferative disorders. Am J Surg Pathol 2000;24:51-5.
Gualco G, Ojopi EB, Chioato L, Cordeiro DL, Negretti F, Bacchi CE. Postsplenectomy sclerosing extramedullary hematopoietic tumor with unexpected good clinical evolution: morphologic, immunohistochemical, and molecular analysis of one case and review of the literature. Appl Immunohistochem Mol Morphol 2010;18:291-5.
Mosley C, Jacene HA, Holz A, Grand DJ, Wahl RL. Extramedullary hematopoiesis on F-18 FDG PET/CT in a patient with metastatic colon carcinoma. Clin Nucl Med 2007;32:878-80.
Qiu D, Hu X, Xu L, Guo X. Extramedullary hematopoiesis on 18F-FDG PET/CT in a patient with thalassemia and nasopharyngeal carcinoma: A case report and literature review. J Cancer Res Ther 2015;11:1034.