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Year : 2012  |  Volume : 8  |  Issue : 1  |  Page : 132-134

Acute leukemic appendicitis in a patient with acute promyelocytic leukemia

Department of Medicine, SUNY Upstate Medical University Syracuse, New York, USA

Date of Web Publication19-Apr-2012

Correspondence Address:
Hatim Karachiwala
Department of Medicine, 750 E Adams Street, Syracuse, NY 13210
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.95194

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 > Abstract 

Leukemic and lymphomatous infiltration of the appendix is a rare complication. We present the case of a 31-year-old male with acute promyelocytic leukemia who developed acute abdomen on day 11 of induction chemotherapy with idarubicin and cytarabine. After appropriate work-up, a clinical diagnosis of acute appendicitis was made. Despite severe pancytopenia, he successfully underwent laparoscopic appendectomy. The final pathology revealed leukemic infiltration of the appendix. It is hypothesized that the leukemic infiltration may play a role in the development of acute appendicitis. Further, this case demonstrates the need to maintain a high index of suspicion and prompt surgical intervention for surgical pathologies in neutropenic patients.

Keywords: Acute promyelocytic leukemia (APL), appendicitis, appendix, leukemic infiltrate

How to cite this article:
Karachiwala H, Das S, Gopaluni S, Gajra A. Acute leukemic appendicitis in a patient with acute promyelocytic leukemia. J Can Res Ther 2012;8:132-4

How to cite this URL:
Karachiwala H, Das S, Gopaluni S, Gajra A. Acute leukemic appendicitis in a patient with acute promyelocytic leukemia. J Can Res Ther [serial online] 2012 [cited 2020 Jul 9];8:132-4. Available from: http://www.cancerjournal.net/text.asp?2012/8/1/132/95194

 > Introduction Top

Acute promyelocytic leukemia (APL) is a hematologic malignancy characterized by malignant transformation of promyelocytes in the marrow. Rarely, the leukemic cells are known to infiltrate the gastrointestinal tract in acute myeloid leukemias (AML). While such tissue infiltration is most commonly described in the monocytic subtypes of AML, it is not reported in APL. It is also not known whether tissue infiltration by leukemic cells is an incidental phenomenon or can actually lead to serious clinical consequences. We describe a patient with acute promyelocytic leukemia who developed acute appendicitis from leukemic infiltration of the appendix. The patient was treated surgically and made a complete recovery from this rare and potentially serious complication.

 > Case Report Top

A 31-year-old Caucasian male presented with history of fatigue and pain in his lower back, radiating around his ribs and sternum of three weeks duration. He initially presented to a community hospital and was noted to be pancytopenic with a total white blood cell count of 0.7 k/ul, hemoglobin of 12 g/dl, hematocrit of 33.5% and a platelet count of 58 k/ul. His absolute neutrophil count on admission was 0.3 k/ul. He developed intermittent high-grade fever and the diagnostic workup for infectious etiology was negative. He was transferred to our facility for further evaluation and management. His history and physical exam was otherwise unremarkable. Complete blood count showed white cell count 0.8 k/ul, hematocrit 27.2%, hemoglobin of 10.9 g/dl, platelet count of 51 k/ul, MCV 86.7 fl, and absolute neutrophil count of 0.21 k/ul. Bone marrow aspirate revealed 95% cellularity and infiltration with sheets of promyelocytes and blasts with Auer rods. Core biopsy was also 95% cellular with sheets of promyelocytes and islands of erythroid precursors and decreased megakaryocytes. Flow cytometry and immune phenotype were done which were consistent with acute promyelocytic leukemia. Flow cytometry revealed expression of mostly myeloid markers and lack of CD34 on the gated cells. FISH for PML and RARA showed translocation (15; 17). Further karyotype analysis showed complex rearrangement of chromosome 3 resulting in partial deletion of 3p and 3q, deletion in the long arm of chromosome 1 and pericenteric inversion in the long arm of chromosome 1. Disseminated intravascular coagulation workup and complete metabolic panel was within normal limits.

He was started on idarubicin (12 mg/m 2 /day on day 1-3) and cytarabine (200 mg/m 2 /day for 7 days) (7+3) induction regimen and all trans-retinoic acid(ATRA 45 mg/m 2 started on day 2) was added. He tolerated chemotherapy well with mild symptoms of nausea and vomiting. He developed neutropenic fever prior to initiation of chemotherapy and was treated with piperacillin-tazobactam and vancomycin per the institution protocol with no identifiable source of infection. He continued to have daily temperature spikes despite being on antimicrobial coverage. On day 11 of induction chemotherapy, he developed vague pain in the left upper quadrant of the abdomen, which progressed to severe left upper quadrant pain along with guarding. His white blood cell count was 0.4 k/ul, platelet count was 24 k/ul, hematocrit was 25.1%, with hemoglobin of 9.1 g/dl and an LDH of 183 units/L. Computerized tomography scan of abdomen and pelvis showed a prominence of the appendix with mild peri- appendiceal fat stranding consistent with early acute appendicitis. He underwent a laparoscopic appendectomy successfully, was continued on antibiotics and received filgrastim support in the peri-surgical period with significant clinical improvement.

Surgical biopsy specimen of the appendix revealed a mononuclear infiltrate in the wall of the appendix extending to the serosa and into the peri-appendiceal fat. The infiltrate composed of scattered large atypical cells with prominent nucleoli, admixed with lymphocytes, histiocytes, and eosinophils [Figure 1] and [Figure 2]. No neutrophils were present. The atypical cells were positive for myeloperoxidase (MPO) [Figure 3] and negative for CD34. CD3 and CD20 highlight scattered T and B-cells, and CD68 stains numerous macrophages consistent with leukemic infiltrate of the appendix. He continues to receive arsenic trioxide per current APL treatment guidelines for his consolidation chemotherapy and is doing well.
Figure 1: Low power showing diffuse leukocytic infi ltrate surrounding typical follicular lymphoid aggregates in the appendiceal wall (× 20)

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Figure 2: High power showing large, atypical cells in the lymphoid aggregate with prominent nucleoli. These cells are admixed with lymphocytes, histiocytes and eosinophils. No neutrophils are present (× 20)

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Figure 3: Immunohistochemistry with anti-myeloperoxidase antibody showing positivity in the atypical mononuclear cells consistent with myeloid lineage (× 20)

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 > Discussion Top

Leukemic and lymphomatous infiltration of the appendix is a rare complication. However, there have been reported cases of acute appendicitis as the initial presentation of some leukemia and lymphoma. [1],[2] AML can involve the gastrointestinal tract, and has been known to also involve the appendix, as demonstrated by prior reported cases, [1],[2],[3] but the involvement of the appendix in the subtype APL is rare. [4],[5] The involvement of extramedullary sites in APL seen in prior cases have been related to relapse of disease, and it is estimated that approximately 3-5% of patients have an extramedullary relapse [4],[5],[6] involving the skin, testes, ear lung, pleura, lymph nodes, mediastinum, thymus, vertebrae, breast, pelvic bones, mandible, gingivae, central nervous system specifically with cases being reported with third cranial nerve palsy and another with involvement of the spinal cord with a paravertebral mass. [4],[5],[7] Other extramedullary site where APL involvement has been reported is specifically the skin, bone, and breast. [4],[8],[9] The prior cases of appendicitis in leukemic patients are associated more with relapsing disease or found to have appendicitis at presentation with no diagnosis of leukemia and lead to poor prognosis, whereas our case is unique in the sense that the patient developed appendicitis on day 11 of chemotherapy and the patient had a good prognosis after surgical intervention. A literature search revealed only one prior case of a patient who presented with APL and was found to have developed appendicitis with leukemic infiltrate on day 11 of induction chemotherapy. [10] It is interesting to note that akin to the prior case, our patient also suffered from appendicitis on day 11 of induction chemotherapy and in both cases the patients were successfully treated with an appendectomy and recovered with no major complications. However, the schedules of the chemotherapy drugs used in induction were marginally different: Our patient received idarubicin (day 1-3) and cytarabine (day 1-7) for induction along with ATRA (day 2), the prior case received ATRA (day 1) and idarubcin (day 2, 4, 6, and 8) for induction therapy. It is unlikely that this variation in schedule would contribute to subsequent complications.

 > Conclusion Top

Leukemic infiltration of the appendix in acute promyelocytic leukemia, presenting as acute appendicitis, is a rare complication. This case is important not only due to its rarity, but also it underscores the importance of timely imaging studies in neutropenic patients with abdominal pain. The patient did not have classic clinical features of acute appendicitis given the left-sided abdominal pain, tenderness and rigidity. It also demonstrates that despite neutropenia these patients significantly benefit from urgent surgical intervention particularly if the leukemic infiltrate in itself is the cause of appendicitis. It is not known whether the leukemic infiltrate is responsible for development of acute appendicitis or additional factors like chemotherapy/ATRA play a role in the pathogenesis.

 > Acknowledgement Top

Dr. Sujhit V. Cherian, for recommending Journal of Cancer Research and Theraputics.

 > References Top

1.Muller G, Dargent J, Duwel V, D'olne D, Vanvuchelen J, Haot J, et al. Leukaemia and lymphoma of the appendix presenting as acute appendicitis or acute abdomen. Four case reports with a review of the Literature. J Cancer Res Clin Oncol 1997;123:560-4.   Back to cited text no. 1
2.Toubai T, Kondo Y, Ogawa T, Imai A, Kobayashi N, Ogasawara M, et al. A case of leukemia of the appendix presenting as acute appendicitis. Acta Haematol 2003;109:199-201.  Back to cited text no. 2
3.Hsiao PJ, Kuo SM, Chen JH, Lin HF, Chu PL, Lin SH, et al. Acute Myelogenous Leukemia and acute Leukemic appendicitis: A case report. World J Gastroenterol 2009;15:5624-5.  Back to cited text no. 3
4.Vega-Ruiz A, Faderl S, Estrov Z, Pierce S, Cortes J, Kantarjian H, et al. Incidence of extramedullary disease in patients with acute promyelocytic leukemia: A single - institution experience. Int J Hematol 2009;89:489-96.  Back to cited text no. 4
5.Speechia G, Lo Coco F, Vignetti M, Avvisati G, Fazi P, Albano F, et al. Extramedullary involvement at relapse in acute promyelocytic leukemia patients treated or not with all- trans retinoic acid: A report by the Gruppo Italiano Malattie Ematologiche dell' Adulto. J Clin Oncolo 2001;19:4023-8.  Back to cited text no. 5
6.Tallman MS. Treatment of relapsed or refractory acute promyelocytic leukemia. Best Pract Res Clin Haematol 2007;20:57-65.  Back to cited text no. 6
7.Rives S, Camos M, Bosch F, Esteve J, Villamor N, Montserrat E. Central nervous system involvement in acute promyelocytic leukemia. A description of two cases and review of the literature. Haematologica 1999;84:473-4.  Back to cited text no. 7
8.Sanz MA, Larrea L, Sanz GF, Martin G, Sempere A, Gomis F, et al. Cutaneous promyelocytic sarcoma at sites of vascular access and marrow aspiration. A characteristic localization of chloromas in acute promyelocytic leukemia? Hematologica 2000;85:758-62.  Back to cited text no. 8
9.Worch J, Ritter J, Fruhwald MC. Presentation of acute promyelocytic leukemia as granulocytic sarcoma. Pediatr Blood Cancer 2008;50:657-60.  Back to cited text no. 9
10.Papageorgiou SG, Foukas P, Economopoulou C, Tsirigotis P, Sakorafas G, Georgakopoulos N, et al. Acute apenditis in patient with acute promyelocytic leukemia. Leuk Res 2011;35:e4-5.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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