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Year : 2019  |  Volume : 62  |  Issue : 4  |  Page : 645-646
Acute myeloid leukemia presenting as fissure-in-ano: A unique case of histologically proven anodermal leukemia cutis

1 Department of Pathology, Lady Hardinge Medical College, New Delhi, India
2 Department of Surgery, Lady Hardinge Medical College, New Delhi, India

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Date of Web Publication14-Oct-2019

How to cite this article:
Gaur K, Puri V, Jain M, Hooda S, Sharma S, Andley M. Acute myeloid leukemia presenting as fissure-in-ano: A unique case of histologically proven anodermal leukemia cutis. Indian J Pathol Microbiol 2019;62:645-6

How to cite this URL:
Gaur K, Puri V, Jain M, Hooda S, Sharma S, Andley M. Acute myeloid leukemia presenting as fissure-in-ano: A unique case of histologically proven anodermal leukemia cutis. Indian J Pathol Microbiol [serial online] 2019 [cited 2023 Sep 30];62:645-6. Available from:


Extramedullary leukemic disease (EMD) is rare and is seen in <10% cases of acute myeloid leukemia (AML).[1] The two commonly described forms of EMD include leukemia cutis (LC) and chloroma.[2] LC is characterized by infiltration of the epidermis, dermis or subcutis by blast cells causing clinically appreciable skin lesions.[2] Anal involvement by leukemic cells is extremely rare. The patient described herein presented with fissure-in-ano. To the best of our knowledge, this is the first described case of histologically proven anodermal LC occurring prior to institution of chemotherapy.

A 50-year- old female presented with complaints of pain and scant perianal discharge since 3 months with an otherwise unremarkable history. Examination revealed an anal fissure at 6 o' clock position accompanied by perianal swelling and induration. Lymphadenopathy or hepatosplenomegaly was not appreciated. The patient's symptoms persisted despite a five day course of ciprofloxacin and conservative measures.

Investigations revealed pancytopenia [Hemoglobin-6.0 g/dl, white blood count-3300 cells/, absolute neutrophil count-1110 cells/, platelet count-70 × 103/μL] with 35% blast cells displaying moderate to abundant cytoplasm [Figure 1]a, few with convoluted nuclei. No Auer rods were seen. The blast cells showed myeloperoxidase, nonspecific esterase (NSE) and Sudan black positivity [Figure 1]b, [Figure 1]c, [Figure 1]d. Histology of the perianal tissue revealed mild papillomatosis and focal epidermal acanthosis with irregular rete ridge elongation. The dermis showed atypical cells with convoluted nuclei, showing a perivascular and periadnexal predilection, focally invading the subcutis. Nucleocytoplasmic ratio was variable in these cells, some appearing histiocytoid [Figure 2]. The cells were strongly positive for MPO, CD 68 [Figure 3] and negative for CD3, HLA-DR, and langerin. Blast markers CD34 and c-kit were negative. However as described previously, these markers are frequently negative in myelomonocytic blast cells.[3] As the patient's platelet count and absolute neutrophil count (ANC) rapidly started falling the next day, bone marrow biopsy was deferred. Blood sugar, viral markers and microbiological tests were unremarkable. A diagnosis of anodermal leukemia cutis (AML-M4) was given. Further evaluation including flow cytometry was not possible as the patient left against medical advice. Acute leukemic transformation in chronic myelomonocytic leukemia could hence not be excluded in this case.
Figure 1: Blast cells in the peripheral smear showing (a) cytoplasmic granules and (b) cytochemical positivity for non-specific esterase (c) Myeloperoxidase negative and (d) positive cells were also noted. [Wrightæs, Nonspecific esterase, Myeloperoxidase, X1000]

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Figure 2: Perianal biopsy showings blasts predominantly in (a) upper dermis and arranged in (b and c) perivascular and (d) periadnexal locations [H&E, 100, 400, X1000]

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Figure 3: Immunohistochemistry showing myeloperoxidase positivity in blasts infiltrating (a) the upper dermis, (b) periadnexal and (c) subcutis (d) CD 68 immunopositivity was also noted in these cells [Myeloperoxidase-DAB, CD68, X100, X200]

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Extramedullary disease (EMD) may precede, develop concurrently or occur after therapy of AML, sometimes presenting as the initial feature of relapse.[2] EMD - AML commonly afflicts lymph nodes and spleen, anal manifestation being exceptionally rare. Histopathological evidence of involvement of the anal/perianal area is essential to document the diagnosis. Herein a perivascular and periadnexal pattern of blast infiltration was seen corroborating earlier descriptions of “leukemia cutis.”[4] Architectural effacement essential to diagnose chloroma was absent in this case.[5] As the neoplastic cells were not exclusively perivascular, extravasation of blasts was excluded.

Cutaneous presentation of myelomonocytic AML masquerading as an anal fissure is exceptionally rare. It is possible that an early infiltration by neoplastic cells weakened the anoderm in our patient, making it more susceptible to fissuring. Wherever feasible, a timely histopathological analysis of a cutaneous biopsy must be performed, as EML is a harbinger of adverse prognosis.[2]

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

Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood 2011;118:3785-93.  Back to cited text no. 1
Solh M, Solomon S, Morris L, Holland K, Bashey A. Extramedullary acute myelogenous leukemia. Blood Rev 2016;30:333-9.  Back to cited text no. 2
Nagra NS, Lozano E, Soilleux E, Mullerat P. A perianal presentation of myeloid sarcoma. BMJ Case Rep 2015;2015:bcr2015209832.  Back to cited text no. 3
Cho-Vega JH, Medeiros LJ, Prieto VG, Vega F. Leukemia cutis. Am J Clin Pathol 2008;129:130-42.  Back to cited text no. 4
Pileri SA, Orazi A, Falini B. Myeloid sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th ed. Lyon: IARC; 2017. p. 167-8.  Back to cited text no. 5

Correspondence Address:
Vandana Puri
Department of Pathology, Lady Hardinge Medical College, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_246_18

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