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Year : 2014  |  Volume : 57  |  Issue : 3  |  Page : 497-498
Intrathoracic extramedullary hematopoiesis in E-beta thalassemia


Department of Hematology, NRS Medical College and Hospital, Kolkata, West Bengal, India

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Date of Web Publication14-Aug-2014
 

How to cite this article:
Mandal PK, Dolai TK. Intrathoracic extramedullary hematopoiesis in E-beta thalassemia. Indian J Pathol Microbiol 2014;57:497-8

How to cite this URL:
Mandal PK, Dolai TK. Intrathoracic extramedullary hematopoiesis in E-beta thalassemia. Indian J Pathol Microbiol [serial online] 2014 [cited 2020 May 29];57:497-8. Available from: http://www.ijpmonline.org/text.asp?2014/57/3/497/138800


Extramedullary hematopoiesis (EMH) is a compensatory phenomenon in hematological diseases with ineffective hematopoiesis, either due to bone marrow replacement disease (e.g., myelofibrosis) or hemolytic anemia with ineffective erythropoesis (e.g., beta thalassemia, sickle cell anemia, and hereditary spherocytosis). [1] Here, we report a case of thalassemia intermedia with intrathoracic EMH.

A 14-year-old boy presented with dry cough for 7 days along with pallor. On query, he had a history of weakness, fatigability, pallor, and jaundice since childhood without any history of blood transfusion or major illness in the past. On examination, he had pallor, jaundice, malar prominence; frontal bossing, splenomegaly (18 cm), and hepatomegaly (6 cm). Laboratory investigations showed hemoglobin 6.3 g/dl, reticulocyte count 3.5%, mean corpuscular volume 72 fl, mean corpuscular hemoglobin 22 pg with normal white blood cell and platelet count. Peripheral blood smear revealed anisocytosis, poikilocytosis, along with target cells and contracted red blood cells. His serum ferritin was 653.2 ng/mL (normal range: 20-150 ng/mL). Liver function tests showed indirect hyperbilirubinemia. Serum tested for hepatitis B surface antigen, anti-hepatitis C virus, and anti-HIV I and II were nonreactive. Hemoglobin electrophoresis (high performance liquid chromatography) showed E-beta thalassemia [Figure 1]. Chest X-ray revealed bilateral paravertebral density with variegated shape [Figure 2]. Magnetic resonance imaging of thorax showed bilateral paravertebral mass without spinal cord involvement [Figure 3]. Computed tomography guided fine-needle aspiration cytology revealed EMH with the presence of megakaryocytes [Figure 4]. Patient responded well to blood transfusion and combined local radiotherapy with hydroxyurea therapy.
Figure 1: High performance liquid chromatography showed E-beta thalassemia (HbA2 = 61.6%, HbF = 33.1%, and HbA0 = 5.3%)

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Figure 2: Chest X-ray revealed bilateral paravertebral density with variegated shape

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Figure 3: Magnetic resonance imaging of thorax showed bilateral paravertebral mass without spinal cord involvement

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Figure 4: Computed tomography guided fine-needle aspiration cytology showed cellular aspirate from extramedullary hematopoiesis mass with the presence of normoblasts (long arrow), myelocytes (short arrow) and megakaryocytes (arrowhead)

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Ineffective red cell production by the bone marrow (ineffective erythropoiesis) forces expansion of the hematopoietic tissue outside the marrow medulla and leads to hematopoietic compensatory involvement, mostly in the form of masses, of other regions in the body - the phenomenon termed EMH. [2] The hematopoietic response in EMH is most often microscopic, but can also result in organomegaly or the development of tumor-like masses, affecting the liver, spleen, and lymph nodes. However, intrathoracic cavity is less frequently involved. [3] EMH can be single or multiple and may present bilaterally. Intrathoracic EMH is usually detected incidentally on chest radiographs. [1] EMH masses are usually asymptomatic, rarely fatal. Sometimes they can spontaneously rupture and produce a hemothorax. [4] EMH is extremely radiosensitive and rapid reduction in the size of the mass can be achieved by small doses of radiation and hydroxyurea, stimulating fetal hemoglobin synthesis has been suggested as an alternative therapeutic approach. [5]

In thalassemia intermedia patients with abnormal chest roentgenogram, intrathoracic EMH must be considered in the differential diagnosis.

 
   References Top

1.
Bobylev D, Zhang R, Haverich A, Krueger M. Extramedullary haematopoiesis presented as intrathoracic tumour in a patient with alpha-thalassaemia. J Cardiothorac Surg 2013;8:120.  Back to cited text no. 1
    
2.
Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: Revisited. Blood Cells Mol Dis 2006;37:12-20.  Back to cited text no. 2
    
3.
Smith P, Manjoney D, Teicher J, Choi KN, Braverman AS. Massive haemothorax due to intrathoracic extramedullary haematopoiesis in a patient with thalassemia intermedia. Chest 1974;65:210-2.  Back to cited text no. 3
    
4.
Chute DJ, Fowler DR. Fatal hemothorax due to rupture of an intrathoracic extramedullary hematopoietic nodule. Am J Forensic Med Pathol 2004;25:74-7.  Back to cited text no. 4
    
5.
Cario H, Wegener M, Debatin KM, Kohne E. Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis. Ann Hematol 2002;81:478-82.  Back to cited text no. 5
    

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Correspondence Address:
Prakas Kumar Mandal
8C/1/N, Roy Para Road, First Floor, Kolkata - 700 050, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.138800

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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