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Table of Contents   
CASE REPORT  
Year : 2014  |  Volume : 7  |  Issue : 1  |  Page : 61-63
Hypoplastic acute myeloid leukemia-report of three cases


1 Department of Pathology, Regional Institute of Medical Sciences, Lamphelpat, Imphal, Manipur, India
2 Department of Pathology, Department, Regional Institute of Medical Sciences, Lamphelpat, Imphal, Manipur, India
3 Arunachal Health Services Department, Regional Institute of Medical Sciences, Lamphelpat, Imphal, Manipur, India

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Date of Web Publication20-Nov-2014
 

   Abstract 

Hypoplastic leukemia, considered as a form of atypical leukemia has been rarely reported in the literatures. It usually affects elderly patients. Even though hypoplastic acute lymphoblastic leukemia has been reported in younger age groups, hypoplastic acute myeloid leukemia (AML) has been rarely reported in children and younger age groups. Here, we report three cases of AML with a hypocellular marrow in younger age groups.

Keywords: Atypical leukemia, hypocellular marrow, hypoplastic leukemia

How to cite this article:
Devi M, Punyabati P, Laishram RS, Khopey J. Hypoplastic acute myeloid leukemia-report of three cases. Ann Trop Med Public Health 2014;7:61-3

How to cite this URL:
Devi M, Punyabati P, Laishram RS, Khopey J. Hypoplastic acute myeloid leukemia-report of three cases. Ann Trop Med Public Health [serial online] 2014 [cited 2019 Nov 13];7:61-3. Available from: http://www.atmph.org/text.asp?2014/7/1/61/145027

   Introduction Top


Acute leukemia presenting with hypocellularity has been known to occur, although rare. The term "hypoplastic leukemia" has been accepted as a different entity. Some authors are of the opinion that hypoplasia could mean a morphologic marker of good prognostic indicator. [1] The occurrence of hypoplastic acute leukemia has been widely recognized as an atypical leukemia. [2] It is characterized by pancytopenia and hypocellularity of the bone marrow (BM) containing equal to or more than 30% blast along with the absence of tissue infiltrates and or tumor masses. [3] Hypoplastic leukemias usually occur in adults. [4] Here, we report three rare cases of hypoplastic acute myeloid leukemia(AML) in young patients.


   Case Reports Top


Case 1

A 17-year-old male presented with fever, pallor, and easy fatigability for 1 month. On examination, he had pallor. Organomegaly or lymphadenopathies were absent. Complete blood count (CBC) showed a hemoglobin of 6.8 g/dl. Total leucocyte count (TLC) of 3000/cumm, differential leukocyte count (DLC) showed P-18%, L-69%, E-3%, myelocytes-3%, atypical cells-7%. Platelets-30,000/cumm. BM aspiration cytology showed markedly hypocellular marrow with the scattered fragment [Figure 1]. Hypocellular (30%), erythropoiesis, myelopoiesis, and megakaryopoiesis were markedly suppressed. DLC showed blast-33%, promyelocyte-2%, myelocytes 2%, polymorphs-10%, E-1%, lymphocytes 50%. Myeloperoxidase (MPO) and Periodic acid-Schiff were negative. A diagnosis of hypoplastic leukemia was given. The lineage whether it was myeloid or lymphoid could not be ascertained. Repeat BM aspiration was done and showed similar findings.
Figure 1: Photomicrograph of bone marrow aspiration smear showing hypocellularity (H and E stain, ×4)

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Patient was referred to a tertiary cancer hospital where BM biopsy was done. BM biopsy showed the presence of blasts with myelofibrosis and increased number of megakaryocytes and suppressed the erythropoiesis. Immunophenotyping was done and showed CD13 positive (89%), CD33 positive (88%), CD117 positive (94%), CD34 positive (86%), HLA-DR positive (88%). However, B cell markers like CD7 (93%) were negative. A diagnosis of AML-M0 (FAB) was made.

Case 2

A 22-year-old female presented with fever, throat pain, and hoarseness of voice for 1 month. On examination, patient was febrile. Organomegaly or lymphadenopathies was absent. CBC showed a hemoglobin of 3.2 g/dl. TLC of 2080/cumm, DLC-showed P-43%, L-48%, M-4%, E-3%, atypical cells-2%. Platelets-40,000/cumm. Erythrocyte sedimentation rate (ESR)-138 mm 1 st h. Red blood cells (RBC) morphology is normochromic and normocytic. Other investigations like liver function test, kidney function test were within normal range. BM aspiration showed markedly hypocellular marrow [Figure 2]. Erythropoiesis and megakaryopoiesis were markedly suppressed. Leukopoiesis showed myeloblast-15%, pormelocyte-26%, myelocyte-5%, metamyelocyte-4%, polymorphs-15%, lymphocytes-35%. MPO was positive. A diagnosis of hypoplastic acute leukemia-AML-M3 was made.
Figure 2: Photomicrograph of bone marrow aspiration smear showing hypocellularity (H and E stain, ×10)

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Case 3

A 10-year-old boy presented with epistaxis, fatigue, and low-grade fever for the last 3 months. Hepatosplenomegaly was detected on examination along with bony tenderness. CBC showed a hemoglobin of 5.2 g/dl. TLC of 2400/cumm, DLC-showed P-30%, L-58%, M-2%, E-2%, atypical cells-8%. Platelets-50,000/cumm. ESR-124 mm 1 st h. RBC morphology is normochromic and normocytic. BM aspiration showed markedly hypocellular marrow. Erythropoiesis and megakaryopoiesis were markedly suppressed. Leukopoiesis showed myeloblast-10%, pormelocyte-24%, myelocyte-16%, metamyelocyte-17%, polymorphs-10%, lymphocytes-23%. MPO was positive. A diagnosis of hypoplastic acute leukemia-AML-M3 was made.


   Discussion Top


The infrequent occurrence of hypoplastic acute leukemia has been recognized as an atypical leukemia defined as hypocellular marrow with ≥20% blasts and no or few blast in the circulating blood. [5] We are reporting three rare cases of hypoplastic AML for the 1 st time in our institute and the state of Manipur. Usually, hypocellular acute lymphoblastic leukemias occur in children. [4] Hypoplastic AML has been rarely reported in pediatric and young patients. However, only two cases of hypoplastic AML have been reported in young patients in India. [6]

The diagnostic criteria have been proposed by Nagai et al. [7] with the following features: Pancytopenia with a rare appearance of blast in peripheral blood; <40% BM cellularity; more than 30% blasts in the BM of all nucleated cells and myeloid phenotypes of leukemic blasts by MPO staining and/or immunophenotyping. All the criteria were fulfilled in our three cases.

Tuzuner et al. revealed a preponderance of FAB-M1 category followed by M2 and M6 types. [8] In a report of two cases in India, all were of M1 subtype. But our first case was M0 subtype, and the other two were of M3 subtype. Literature regarding M3 subtype is very rare though Kojima et al. [9] reported hypocellular acute promyelocytic leukemia with a tetraploid clone.

Though the pathogenesis of hypocellularity still remains speculative, it seems clear that these patients bear a lower leukemic burden and experience a more indolent course and can commonly achieve a good response to remission induction therapy. [1],[10] However, two mechanisms have been proposed. First, it has been suggested that leukemia cell populations inhibit myelopoiesis through a humoral mechanism. [11] Second, an increased susceptibility of myeloid precursors to the inhibitor in older patients might play a role in the genesis of hypoplasia. [1] Recent reports suggest the beneficial effects of granulocyte colony-stimulating factor in the hematopoietic reconstruction following chemotherapy. [12]


   Conclusion Top


Hypoplastic AML can occur in young patients though rare. Larger study is the need of the hour to bring into light the exact prevalence of this condition.

 
   References Top

1.
Beard ME, Bateman CJ, Crowther DC, Wrigley PF, Whitehouse JM, Fairley GH, et al. Hypoplastic acute myelogenous leukaemia. Br J Haematol 1975;31:167-76.  Back to cited text no. 1
    
2.
Wei SY, Lin SF, Chen TP, Liu HW, Chang CS, Lin TC. Remission of hypoplastic acute leukemia by low dose Ara-C: One case report. Gaoxiong Yi Xue Ke Xue Za Zhi 1991;7:531-5.  Back to cited text no. 2
    
3.
de Bock R, de Jonge M, Korthout M, Wouters E, van Bockstaele D, van der Planken M, et al. Hypoplastic acute leukemia: Description of eight cases and search for hematopoietic inhibiting activity. Ann Hematol 1992;65:247-52.  Back to cited text no. 3
    
4.
Matloub YH, Brunning RD, Arthur DC, Ramsay NK. Severe aplastic anemia preceding acute lymphoblastic leukemia. Cancer 1993;71:264-8.  Back to cited text no. 4
    
5.
Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood 2002;100:2292-302.  Back to cited text no. 5
    
6.
Jain D, Singh T, Kumar N. Hypocellular acute myeloid leukemia with bone marrow necrosis in young patients: Two case reports. J Med Case Rep 2009;3:27.  Back to cited text no. 6
    
7.
Nagai K, Kohno T, Chen YX, Tsushima H, Mori H, Nakamura H, et al. Diagnostic criteria for hypocellular acute leukemia: A clinical entity distinct from overt acute leukemia and myelodysplastic syndrome. Leuk Res 1996;20:563-74.  Back to cited text no. 7
    
8.
Tuzuner N, Cox C, Rowe JM, Bennett JM. Hypocellular acute myeloid leukemia: The Rochester (New York) experience. Hematol Pathol 1995;9:195-203.  Back to cited text no. 8
    
9.
Kojima K, Imaoka M, Noguchi T, Narumi H, Uchida N, Sakai I, et al. Hypocellular acute promyelocytic leukemia with a tetraploid clone characterized by two t(15;17). Cancer Genet Cytogenet 2003;145:169-71.  Back to cited text no. 9
    
10.
Needleman SW, Burns CP, Dick FR, Armitage JO. Hypoplastic acute leukemia. Cancer 1981;48:1410-4.  Back to cited text no. 10
    
11.
Quesenberry PJ, Rappeport JM, Fountebouni A, Sullivan R, Zuckerman K, Ryan M. Inhibition of normal murine hematopoiesis by leukemic cells. N Engl J Med 1978;299:71-5.  Back to cited text no. 11
    
12.
Lee M, Chubachi A, Niitsu H, Miura I, Yanagisawa M, Hirokawa M, et al. Successful hematopoietic reconstitution with granulocyte colony-stimulating factor in a patient with hypoplastic acute myelogenous leukemia. Intern Med 1995;34:692-4.  Back to cited text no. 12
    

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Correspondence Address:
Rajesh Singh Laishram
Department of Pathology, Regional Institute of Medical Sciences, Lamphelpat, Imphal - 795 004, Manipur
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1755-6783.145027

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