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Year : 2014  |  Volume : 57  |  Issue : 4  |  Page : 638-639
Co-existence of acute myeloid leukemia infiltration and extramedullary hematopoiesis in appendix

1 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
3 Department of Surgical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

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Date of Web Publication11-Oct-2014

How to cite this article:
Vasudevan JA, Nair RA, Prem S, Nair CK. Co-existence of acute myeloid leukemia infiltration and extramedullary hematopoiesis in appendix . Indian J Pathol Microbiol 2014;57:638-9

How to cite this URL:
Vasudevan JA, Nair RA, Prem S, Nair CK. Co-existence of acute myeloid leukemia infiltration and extramedullary hematopoiesis in appendix . Indian J Pathol Microbiol [serial online] 2014 [cited 2023 Nov 30];57:638-9. Available from:

The present case is that of a 29-year-old male who presented with abdominal discomfort and pain in the legs in a local hospital. Ultrasound scan of abdomen was with in normal limits. Routine peripheral smear examination revealed acute myeloid leukemia and was referred to our centre. Physical examination revealed anaemia. No lymphadenopathy or hepatosplenomegaly was present. Perepheral smear examination confirmed acute myeloid leukemia. Bone marrow studies with flowcytometry were done. Bone marrow smears showed 82% blasts including promonocytes. Blasts were peroxidise positive by cytochemistry and were positive for CD 13, CD 33, CD 64, CD 11c, CD 14, HLA DR by flowcytometry. A diagnosis of acute monocytic leukaemia was made. The patient underwent induction chemotherapy with three doses of cytosine arabinoside and daunorubicin. After third dose of induction chemotherapy, patient complained of abdominal pain with tenderness of right iliac fossa. Ultrasound scan was suggestive of acute appendicitis. Emergency appendicectomy was done. Intraoperatively appendix was inflamed. No pus, ascites or meckel's diverticulum was found. Gross examination showed congestion on the external surface and thickened wall. Microscopic examination showed blasts infiltrating the bowel wall admixed with several megakaryocytes [Figure 1]. Histopathological features of acute appendicitis were not observed. Immunohistochemistry done showed positivity of blasts for CD 11 c and CD 33 [Figure 2]a-b. Megakaryocytes were positive for CD61 [Figure 2]c. Presence of blasts admixed with megakaryocytes suggests extramedullary hematopoiesis. To exclude a chronic myeloproliferative neoplasm, molecular studies for BCR- ABL 1 fusion gene was done but was negative. Our review of literature revealed a case report of extramedullaryhematopoiesis in appendix as a rare complication of primary myelofibrosis. [1] Molecular studies for JAK2 could not be done in our case but clinically there was no hepatosplenomegaly. Extramedullaryhemopoiesis has been rarely described in appendix. Proposed theories about the pathogenesis of extramedullaryhematopoiesis include extrusion of cells of bonemarrow secondary to bonemarrow fibrosis or replacement by other cells, reactivation in those organs where hematopoiesis occurred in embryonic and fetal life, embolisation of hematopoietic stem cells to different organs. [2] We are reporting this case because of the unusual co-existence of both extramedullary hematopoiesis and acute myeloid leukemia infiltration in appendix which is not yet reported in literature.
Figure 1: (a) Blasts admixed with megakaryocytes infiltrating the appendiceal wall (H and E, × 100), (b) Megakaryocytes admixed with blasts. (H and E, ×400), (c) Blasts infiltrating the appendiceal wall. (H and E, ×400), (d) Blasts showed moderate cytoplasm and convoluted nucleus. (H and E, ×1000)

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Figure 2: (a) Blasts were positive for CD 11c. (IHC, ×400), (b) Blasts were positive for CD 33. (IHC, ×400), (c) Megakaryocytes were positive for CD 61. (IHC, ×400)

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   References Top

Elpek GO, Bozova S, Erdogan G, Temizkan K, Ogus M. Extramedullary hematopoiesis mimicking acute appendicitis: A rare complication of acute myelofibrosis. Virchows Arch 2006;449:258-61.  Back to cited text no. 1
Dey P, Varma S, Varma N. Fine needle aspiration biopsy of extramedullaryleukemia. Acta Cytol 1996;40:252-6.  Back to cited text no. 2

Correspondence Address:
Rekha A Nair
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram - 695 011, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.142719

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