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Year : 2022  |  Volume : 37  |  Issue : 3  |  Page : 293-294  

A curious case of suspicious lymphadenopathy in a hereditary spherocytosis patient reported as extramedullary hematopoiesis


Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India

Date of Submission12-Jan-2022
Date of Decision05-Mar-2022
Date of Acceptance16-Mar-2022
Date of Web Publication02-Nov-2022

Correspondence Address:
Dr. Rakesh Kumar
Department of Nuclear Medicine, Division of Diagnostic Nuclear Medicine, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijnm.ijnm_10_22

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   Abstract 


We present a case of a 23-year-old male patient with complaints of fever, cough, and persistent anemia for the past 6 months and with a known history of hereditary spherocytosis. Computed tomography (CT) thorax demonstrated multiple paravertebral lesions in the bilateral thoracic cavities, suggestive of lymphadenopathy; subsequently, 18Flurodeoxyglucose PET/CT was done with suspicion of lymphoma, which showed no significant metabolic activity in those lesions. Thus, in view of clinical and metabolic status, lesions were considered extramedullary hematopoiesis (EMH). This case highlights the importance of considering EMH, while interpreting suspicious lymphadenopathy in cases of chronic anemia and also possible scan findings in the same.

Keywords: Anemia, extramedullary hematopoiesis, FDG PET/computed tomography, lymphoma


How to cite this article:
Khan D, Sharma A, Sagar S, Thayumanavan T, Kumar R. A curious case of suspicious lymphadenopathy in a hereditary spherocytosis patient reported as extramedullary hematopoiesis. Indian J Nucl Med 2022;37:293-4

How to cite this URL:
Khan D, Sharma A, Sagar S, Thayumanavan T, Kumar R. A curious case of suspicious lymphadenopathy in a hereditary spherocytosis patient reported as extramedullary hematopoiesis. Indian J Nucl Med [serial online] 2022 [cited 2022 Nov 29];37:293-4. Available from: https://www.ijnm.in/text.asp?2022/37/3/293/360251



A 23-year-old male patient presented with fever, cough, and persistent anemia for the past 6 months not subsiding with medication. On clinical examination, he had kyphoscoliosis and evidence of anemia signs. He was a known case of hereditary spherocytosis (with splenectomy done 10 years back) and type-1 diabetes mellitus (on insulin therapy). Computed tomography (CT) scan of the thorax showed multiple paravertebral lesions in bilateral thoracic cavities suggestive of lymphadenopathy [Figure 1]a and [Figure 1]b. With a suspicion of lymphoma, the patient underwent 18F-FDG PET/CT, which showed multiple lesions in the bilateral paravertebral region from D7 to D10 vertebrae (largest measuring 3 cm × 2.6 cm × 3.2 cm), with no significant metabolic activity [Figure 1]c, [Figure 1]d, [Figure 1]e. In view of the metabolic status and clinical history, lesions were considered extramedullary hematopoiesis (EMH).
Figure 1: (a and b) With a suspicion of lymphoma, the patient underwent 18F-FDG PET/computed tomography, which showed multiple lesions in bilateral paravertebral region from D7 to D10 vertebrae (largest measuring 3 cm × 2.6 cm × 3.2 cm) with no significant metabolic activity (c-e). In view of the metabolic status and clinical history, lesions were considered extramedullary hematopoiesis

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EMH refers to the production of blood cells outside the bone marrow, commonly in liver, spleen, kidney, and lymph nodes, while intrathoracic cavity is less frequently involved. It is commonly seen in myelofibrosis (most common), leukemia, sickle cell disease, diffuse osseous metastatic disease replacing the bone marrow, and thalassemia.[1],[2] The most commonly used diagnostic methods for EMH are magnetic resonance imaging and CT scans.[3],[4] On CT, EMH typically appears as heterogeneous, hypovascular soft tissue masses, often interspersed with areas of fat attenuation.[4] In PET/CT, EMH lesions demonstrate mild-to-moderate metabolic activity when compared with malignant lesions, SUVmax value is generally low, and the tissue appears normal. Underlying hematopoietic disorder may suggest EMH, and a sampling of this tissue confirms the diagnosis.[5] However, risk of hemorrhage cannot be avoided during sampling, particularly in EMH, as it is an invasive procedure. An adequate noninvasive workup may be clinically useful in the evaluation of the paravertebral masses.[6] There have been only limited data records of PET/CT detecting EMH; all of these cases were in patients with different type of malignant diseases and characteristically exhibited mild FDG avidity (low SUVmax) compared to the malignancies with high FDG uptake.[5],[7],[8],[9],[10] Hence, EMH has to be considered as differential while reporting paraveretebral masses in patients with a history of chronic anemia or myeloproliferative disorders. Our case demonstrated incidental detection of EMH in a chronic anemia patient while investigating for suspicious lymphadenopathy on anatomic imaging. These patients are usually asymptomatic but can cause symptoms on compression of neighboring structures, in which case radiation therapy or surgical interventions are required; otherwise, EMH usually regresses after treatment with blood transfusions and hydroxyurea.[11]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Roberts AS, Shetty AS, Mellnick VM, Pickhardt PJ, Bhalla S, Menias CO. Extramedullary haematopoiesis: Radiological imaging features. Clin Radiol 2016;71:807-14.  Back to cited text no. 1
    
2.
Debard A, Demasles S, Camdessanché JP, Duband S, Mohammedi R, Antoine JC. Dural localization of extramedullary hematopoiesis. Report of a case. J Neurol 2009;256:837-8.  Back to cited text no. 2
    
3.
Chunduri S, Gaitonde S, Ciurea SO, Hoffman R, Rondelli D. Pulonary extramedullary hematopoiesis in patients with myelofibrosis undergoing allogeneic stem cell transplantation. Haematologica 2008;93:1593-5.  Back to cited text no. 3
    
4.
Fucharoen S, Winichagoon P. Clinical and hematologic aspects of hemoglobin E beta-thalassemia. Curr Opin Hematol 2000;7:106-12.  Back to cited text no. 4
    
5.
Paydaş S, Sargın Ö, Gönlüşen G. PET CT imaging in extramedullary hematopoiesis and lung cancer surprise in a case with thalassemia intermedia. Turk J Haematol 2011;28:60-2.  Back to cited text no. 5
    
6.
Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia. Eur Spine J 2010;19:871-8.  Back to cited text no. 6
    
7.
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.  Back to cited text no. 7
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8.
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.  Back to cited text no. 8
    
9.
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.  Back to cited text no. 9
    
10.
Seo M, Kim H, Jo JC, Choi Y, Cha HJ, Lim JH, et al. Mass-forming extramedullary hematopoiesis in multiple myeloma: 18F-FDG PET/CT is useful in excluding extramedullary myeloma involvement. Tumori 2016;102:116-8.  Back to cited text no. 10
    
11.
Emamhadi M, Alizadeh A. Effect of hypertransfusion on extramedullary hematopoietic compression mass in thalassemia major: A case report. Iran J Radiol 2012;9:154-6.  Back to cited text no. 11
    


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