Journal of Cytology
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 Table of Contents    
Year : 2021  |  Volume : 38  |  Issue : 2  |  Page : 109-110
Extramedullary hematopoiesis in lymphangioma - A cytological study

1 Department of Pathology, Maulana Azad Medical College, New Delhi, India
2 Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi, India

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Date of Submission24-Dec-2020
Date of Decision24-Mar-2021
Date of Acceptance29-Mar-2021
Date of Web Publication15-May-2021

How to cite this article:
Kushwaha P, Singh M, Jain S, Neogi S. Extramedullary hematopoiesis in lymphangioma - A cytological study. J Cytol 2021;38:109-10

How to cite this URL:
Kushwaha P, Singh M, Jain S, Neogi S. Extramedullary hematopoiesis in lymphangioma - A cytological study. J Cytol [serial online] 2021 [cited 2022 Nov 29];38:109-10. Available from:

Dear Editor,

Extramedullary hematopoiesis (EMH) is the development and growth of hematopoietic tissue outside the bone marrow. It usually occurs as a compensatory mechanism to sustain hematopoiesis in response to any alteration in the normal production of blood cells.[1]

A 1-year-old male child presented with a slowly increasing neck mass for 4 months. On examination, a soft cystic swelling was noted on the left side of the neck, 7 cm × 4 cm in size, non-tender [Figure 1]a. Ultrasonography (USG) neck showed a solid cystic lesion and was referred for fine-needle aspiration cytology (FNAC). It was performed using a 23-gauge needle attached with a disposable 20 mL syringe under aseptic precautions as per the standard technique. FNAC from solid areas yielded a fluid aspirate. Multiple air-dried smears were prepared and stained with the Giemsa stain. Smears showed mature lymphoid cells, hemosiderin-laden macrophages, numerous immature myeloid cells (myelocytes, metamyelocytes), and nucleated Red blood cells (RBC) in a proteinaceous background [Figure 1]b,[Figure 1]c, [Figure 1]d. Hemogram was within normal limits and peripheral smear showed no shift to the left in the leucocytes and absence of nucleated RBC. CT angiography was performed which revealed the neck mass to be a lymphangioma. Based on the cytological, hematological, and radiological findings, diagnosis of the extramedullary hematopoiesis in lymphangioma was made. USG abdomen showed no evidence of lymphadenopathy/hepatosplenomegaly. The swelling was treated with sclerotherapy, following which the swelling markedly reduced in size and maintained over a 6-month follow-up. The patient is still kept under regular follow-up to ensure detection and earliest treatment of any associated cause for extramedullary hematopoiesis.
Figure 1: (a) Clinical picture showing soft cystic swelling on left side of neck (arrow). (b) FNAC smear showing erythroid precursor (blue arrow) and myeloid precursor (green arrow) (MGG 400X). (c) FNAC smear showing erythroid precursor at a higher magnification (MGG 1000X). (d) FNAC smear showing myeloid precursor at a higher magnification (MGG 1000X)

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EMH is usually caused due to ineffective hematopoiesis in the bone marrow. It may be associated with diseases like chronic anemia, sickle cell disease, thalassemia, spherocytosis, hematological malignancies such as myelofibrosis, leukemias, myeloproliferative disorders, metastatic carcinomas in bone marrow, and underlying malignancies.[2] Although infrequent, cases of EMH have been reported in which no underlying hematolymphoid disorder/malignancy is noted; as in the present case.[3],[4] Fan et al.[3] described the term 'Idiopathic EMH' for such cases and supported that simple monitoring and follow-up of the patient might be adequate.

The diagnosis of EMH requires identification of the myeloid, erythroid/megakaryocytic precursors. Usually, the diagnosis can be made on morphology alone; however, immunohistochemical stains, such as glycophorin, myeloperoxidase, and CD61 could be used to highlight the multilineage hematopoietic elements (erythroid, myeloid, and megakaryocytic elements, respectively).[5]

FNAC in conjunction with clinical history and hematological findings usually helps to diagnose this entity correctly.[6] But only occasional case reports and two case series of EMH diagnosed on cytology have been reported yet.[1],[5],[6]

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

   References Top

Sheikh U, Rodic N, Maleki Z. Extramedullary hematopoiesis: Cytomorphologic, histologic, and radiologic findings in sixteen cases. Acta Cytol 2015;59:144-8.  Back to cited text no. 1
Kishore M, Kaushal M, Marwah S, Sharma M, Sharma N, Mittal V. Cytodiagnosis of extramedullary hematopoiesis in thyroid gland unravelling an asymptomatic hematological malignancy. Diagn Cytopathol 2019;47:320-4.  Back to cited text no. 2
Fan N, Lavu S, Hanson CA, Tefferi A. Extramedullary hematopoiesis in the absence of myeloproliferative neoplasm: Mayo Clinic case series of 309 patients. Blood Cancer J 2018;8:119.  Back to cited text no. 3
Koch CA, Li C-Y, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: Associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc 2003;78:1223-33.  Back to cited text no. 4
Hudson JB, Murad FM, Kunkel JE, Collins BT. Endoscopic ultrasound guided fine-needle aspiration of a splenic hemangioma with extramedullary hematopoiesis. Diagn Cytopathol 2013;41:1086-90.  Back to cited text no. 5
Kapatia G, Kaur A, Rastogi P, Sreedharanunni S, Gupta P, Rohilla M, et al. Extramedullary hematopoiesis: Clinical and cytological features. Diagn Cytopathol 2020;48:191-6.  Back to cited text no. 6

Correspondence Address:
Dr. Meeta Singh
Department of Pathology, Maulana Azad Medical College, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JOC.JOC_206_20

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