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  Table of Contents    
LETTER TO EDITOR  
Year : 2018  |  Volume : 61  |  Issue : 3  |  Page : 464-467
Coexistence of T-cell lymphoblastic lymphoma and acute myeloid leukemia mimicking acute lymphocytic leukemia


1 Department of Clinical Laboratory, Affiliated Hospital of Engineering University of Hebei, Hebei Province, China
2 Department of Hematology, Affiliated Hospital of Engineering University of Hebei, Hebei Province, China
3 Department of Science and Education, Affiliated Hospital of Engineering University of Hebei, Hebei Province, China
4 Department of Obstetrics, Affiliated Hospital of Engineering University of Hebei, Hebei Province, China

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Date of Web Publication13-Jul-2018
 

How to cite this article:
Liu K, Zhou F, Fu Z, Yin J, Li W. Coexistence of T-cell lymphoblastic lymphoma and acute myeloid leukemia mimicking acute lymphocytic leukemia. Indian J Pathol Microbiol 2018;61:464-7

How to cite this URL:
Liu K, Zhou F, Fu Z, Yin J, Li W. Coexistence of T-cell lymphoblastic lymphoma and acute myeloid leukemia mimicking acute lymphocytic leukemia. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Oct 15];61:464-7. Available from: http://www.ijpmonline.org/text.asp?2018/61/3/464/236620




Editor,

A 78-year-old man presented with T-cell lymphoblastic lymphoma (T-LBL) of the right cervical lymph node coexisting with acute myeloid leukemia (AML) and mimicking acute lymphocytic leukemia (ALL). The histopathology findings of biopsy specimens in the cervical lymph node indicated T-LBL. Bone marrow (BM) aspiration revealed AML with ALL-like morphologic features. Thus, we called the condition AML mimicking ALL. The patient was diagnosed with T-LBL and simultaneous AML. To date, no case reports of a patient diagnosed with simultaneous T-LBL and AML with ALL-like morphologic features have been reported. We present the case given its rarity, diagnostic difficulty, and aggressive course. This case illustrates the importance of recognizing the clinical features and avoiding misdiagnosis. Clinical trials are available to further investigate how to prolong survival time and improve the prognosis for patients suffering from T-cell lymphoproliferative disorder and AML simultaneously.[1]

A previously healthy 78-year-old man sought medical attention in April 2016 with a 10-day history of painless, progressive right cervical masses, and light pain in his upper left abdomen. Palpation identified multiple enlarged lymph nodes of different sizes in the neck, groin, and armpits with the larger well-demarcated masses approximately 3 cm × 2 cm behind the right sternocleidomastoids. On physical examination, the doctor observed painless red papula covering his skin, especially the skin of his chest and back. Splenomegaly, a small pleural effusion, enlarged intraperitoneal cavity, and retroperitoneal nodes were detected by computed tomography. Laboratory findings were as follows: white blood cell count was 94.39 × 109/l, red blood cell count was 1.76 × 1012/l, hemoglobin was 64 g/l, platelet count was 38 × 109/l, carbohydrate antigens CA125 was 42.2 U/ml (normal value 0–30.2 U/ml), and human immunodeficiency virus was negative. Peripheral blood smear revealed approximately 88% blast cells [Figure 1]a. Subsequently, BM aspirate revealed the blasts accounted for 93% of all nucleated cells [Figure 1]b. The blasts were different sizes, with round to oval nuclei, fine nuclear chromatin, scant blue cytoplasm, and with or without distinct nucleoli. The blasts were negative for myeloperoxidase (MPO). The blasts were supported with ALL-like morphologic and primary cytochemical staining features. Immunophenotypic analysis of BM aspirate by flow cytometry confirmed the abnormal cell population with 89.12% of nucleated cells. The cell population was positive for HLA-DR, CD7, CD33, CD123, CD117, and terminal deoxynucleotidyl transferase (TDT); a few cells expressed CD56 and CD11C; the cell population was negative for CD34, CD38, CD64, CD36, CD14, CD4, CD2, MPO, cCD79a, cCD3, CD16, CD8, CD99, CD5, and CD13 [Figure 2] and [Figure 3]. The results of the immunophenotypic analysis suggested AML. Lymph node biopsy of the right cervical indicated diffuse proliferation of medium-sized lymphocytes with fine chromatin and scarce cytoplasm [Figure 1]c and [Figure 1]d, and immunohistochemical stain revealed that the lymphoma cells were focally positive for CD20 and CD3, positive for CD7, TDT, and Ki67 (40%–50%), and negative for CD5, MPO, CD21, CD23, CD10, Bc-6, and cyclin-D1 [Figure 1] CD3 and TDT]. The histopathological findings of biopsy specimens of the right cervical lymph node indicated T-LBL. To the best of our knowledge, the simultaneous coexistence of T-LBL and AML is very rare. Given that the BM aspirate suggested ALL-like morphologic and primary cytochemical staining features, we repeated BM aspirate immunophenotypic analysis, and the result still supported AML. Cytogenetic analysis of the BM cells using the G and R banding technique revealed a 46, XY(20) karyotype. WT1 gene expression analysis by fluorescent quantitative polymerase chain reaction and the TaqMan probe method revealed that 0.03% of the cells were positive for WT1 gene expression (WT1-positive standard: >2% of positive cells). The patient did not harbor FLT3 mutations. Unfortunately, we did not have the opportunity to study other fusion genes. Based on morphoscopic and cytochemical staining features and immunophenotypic analysis by flow cytometry, we thought that the patient met M0 diagnostic criteria proposed by FAB in 1991.[2] The findings led to a diagnosis of T-LBL Stage IV and simultaneous AML according to the World Health Organization (WHO) classification.[3]
Figure 1: (a) Peripheral blood smear revealed increased blasts (Wright-Giemsa, ×1000). (b) Morphology of bone marrow aspiration revealed that the blasts exhibited different sizes with round to oval nuclei, fine nuclear chromatin, scant blue cytoplasm, and with or without distinct nucleoli. (Wright-Giemsa, ×1000). (c and d) Lymph node biopsy and immunohistochemistry analyses of the right cervical lymph node. Diffuse proliferation of medium-sized lymphocytes with fine chromatin and scarce cytoplasm (c: H and E, ×100; d: H and E, ×200). These cells expressed CD3 (original magnification: ×200) and terminal deoxynucleotidyl transferase (original magnification: ×100)

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Figure 2: Bone marrow aspirate immunophenotypic analysis by flow cytometry confirmed an abnormal cell population with 89.12% nucleated cells. The cell population was positive for HLA-DR, CD7, CD117, and CD33. The cell population was negative for myeloperoxidase, cCD3, CD34, and CD13

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Figure 3: Bone marrow aspirate immunophenotypic analysis by flow cytometry confirmed an abnormal cell population with 89.12% nucleated cells. A few blasts expressed CD56. The cell population was negative for CD34, CD19, CD20, CD4, cCD3, CD99, CD5, and CD2

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We started treatment with the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) plan after the diagnosis. The enlarged cervical lymph nodes obviously shrank after 8 days of chemotherapy. However, BM aspirate examination indicated no marked improvement in blast percentage. Subsequent chemotherapy was postponed given that the patient presented with pancytopenia and hematologic recovery was slow and insufficient. The patient was discharged on request due to his family's financial status. In June 2016, the patient came back again due to enlarged lymph nodes and multiple maculopapules in the trunk. Treatment was immediately initiated using the CHOP-VP16 plan. Enlarged lymph node and rashes subsided significantly after 3 weeks. Although the patient experienced BM blast reductions of >40% with treatment, BM examination revealed that the patient was not in remission. Agranulocytosis, upper respiratory infection, and high fever appeared during chemotherapy, so the patient was administered leukogenic and anti-infective treatment. The patient was discharged on request again when he got better. However, the disease gradually became difficult to control. The patient died 6 months after initial diagnosis as a result of further disease progression.

Several reports of the coexistence of T-cell lymphoproliferative disorder and AML before any form of treatment have been reported. These reported AML cases exhibited typical morphoscopic and cytochemical staining features. We herein describe an elderly man with T-LBL simultaneously occurring with AML mimicking ALL. Regarding the disease, our patient had no history of immunodeficiency or taking immunosuppressant medications. Thus, we considered that T-LBL was not diagnosed until the onset of AML. Furthermore, although enlarged lymph nodes and rashes subsided significantly after chemotherapy, BM examination consistently revealed that the patient was not in remission. This clinical history suggested that the onset of AML was independent of T-LBL and potentially worsened the latent T-LBL. Reasonable explanations for the coexistence of AML and T-LBL include the following. It is possible that both diseases shared a common mechanism and changes in pluripotent hematopoietic stem cells. In addition, some researchers suggested that both AML and T-LBL are rapidly progressive diseases, and the coexistence of AML and T-LBL was just a coincidence.

Based on our extensive review of the literature, we did not identify another case of a patient diagnosed with simultaneous T-LBL and AML with ALL-like morphologic and primary cytochemical staining features. We considered the patient unfit for intensive chemotherapy given his old age, serious complications, and high percentage of BM blast cells. We therefore decided to administer chemotherapy indicated for ALL. The patient's enlarged lymph nodes and rashes subsided significantly after the chemotherapy. However, the treatment for T-LBL was insufficient for the treatment of AML. Thus, BM examination consistently revealed that the patient was not in remission. The patient died 6 months after the initial diagnosis as a result of further disease progression. This clinical history also allowed us to determine the correct diagnosis for our patient. Early T-cell precursor (ETP) ALL is a high-risk subgroup of T-lineage ALL characterized by specific stem cell and myeloid features.[4] As a T-lineage ALL, immunophenotype of the leukemic cells must be positive for cCD3 and negative for MPO.[5] However, the blasts of our patient were negative for cCD3. According to the WHO classification, acute undifferentiated leukemia (AUL) shows no lineage-specific antigens.[6] However, the blasts of our patient were positive for CD33 and CD117, which are the typical myeloid markers. Thus, ETP-ALL and AUL were ruled out in the differential diagnosis of AML. Some researchers have demonstrated that T-cell receptor (TCR)-delta gene rearrangement studies can aid in establishing the correct diagnosis of T-cell ALL.[7] Therefore, TCR-delta gene rearrangements studies suggested the true nature of the blasts in the BM smear from our patient indirectly. The expression of TCR-delta gene rearrangements should be performed to determine the diagnosis of acute leukemia in the future. At present, the goals of treatment for elderly patients with AML seemed to favor better disease control and quality of life improvement over complete remission achievement. Researchers have made progress in AML treatments for elderly patients. In addition to T-LBL treatment and providing the best supportive care, the patient can benefit from medication for the treatment of AML, such as low-dose cytarabine, sapacitabine, and gene-targeting drugs.[8],[9],[10]

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.

Financial support and sponsorship

Foundation of Health and family planning Commission of Hebei No. 20180804.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

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Tokuhira M, Hanzawa K, Watanabe R, Sekiguchi Y, Nemoto T, Toyozumi Y, et al. Co-existence of acute myeloid leukemia with multilineage dysplasia and Epstein–Barr virus-associated T-cell lymphoproliferative disorder in a patient with rheumatoid arthritis: A case report. J Hematol Oncol 2009;2:27.  Back to cited text no. 1
    
2.
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, et al. Proposal for the recognition of minimally differentiated acute myeloid leukaemia (AML-MO) Br J Haematol 1991;78:325-9.  Back to cited text no. 2
    
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Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the world health organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127:2391-405.  Back to cited text no. 3
    
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Neumann M, Heesch S, Schlee C, Schwartz S, Gökbuget N, Hoelzer D, et al. Whole-exome sequencing in adult ETP-ALL reveals a high rate of DNMT3A mutations. Blood 2013;121:4749-52.  Back to cited text no. 4
    
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Borowitz MJ, Chan JK, Bene MC, Arber DA, et al. Early T-cell precursor lymphoblastic leukaemia. In: Steven H. Swerdlow, Elias Campo, Nancy Lee Harris, Elaine S. Jaffe, Stefano A. Pileri, Harald Stein, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised. 4th ed. 69372 Lyon Cedex 08, France: IARC Press; 2017. p. 212.  Back to cited text no. 5
    
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Borowitz MJ, Bene MC, Harris NL, Porwit A, Matutes E, Arber DA, et al. Acute leukaemias of ambiguous lineage. In: Steven H. Swerdlow, Elias Campo, Nancy Lee Harris, Elaine S. Jaffe, Stefano A. Pileri, Harald Stein, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised. 4th ed. 69372 Lyon Cedex 08, France:IARC Press; 2017. p. 180-7.  Back to cited text no. 6
    
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Natarajan S, Nancy NK, Kamalalayam R, Thangarajan R, et al. T Cell Receptor Gamma and Delta Gene Rearrangements in T-cell Acute Lymphoblastic Leukemia in South India and Quantitation of Minimal Residual Disease. Nature Precedings; 2010.  Back to cited text no. 7
    
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Kantarjian HM, Thomas XG, Dmoszynska A, Wierzbowska A, Mazur G, Mayer J, et al. Multicenter, randomized, open-label, phase III trial of decitabine versus patient choice, with physician advice, of either supportive care or low-dose cytarabine for the treatment of older patients with newly diagnosed acute myeloid leukemia. J Clin Oncol 2012;30:2670-7.  Back to cited text no. 8
    
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Burnett AK, Milligan D, Prentice AG, Goldstone AH, McMullin MF, Hills RK, et al. A comparison of low-dose cytarabine and hydroxyurea with or without all-trans retinoic acid for acute myeloid leukemia and high-risk myelodysplastic syndrome in patients not considered fit for intensive treatment. Cancer 2007;109:1114-24.  Back to cited text no. 9
    
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Kantarjian H, Faderl S, Garcia-Manero G, Luger S, Venugopal P, Maness L, et al. Oral sapacitabine for the treatment of acute myeloid leukaemia in elderly patients: A randomised phase 2 study. Lancet Oncol 2012;13:1096-104.  Back to cited text no. 10
    

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Correspondence Address:
Wei Li
Department of Obstetrical, Affiliated Hospital of Engineering University of Hebei, Congtai Street Number 81, HanDan, Hebei Province
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_6_18

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