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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 2  |  Page : 257-259
Transient leukemia

Department of Haematology, All India Institute of Medical Sciences, New Delhi - 110 029, India

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A new born with a mongoloid slant, brachycephaly and low-set ears presented at birth with a total leucocyte count of 57 x 10 3 /無 and the differential leucocyte count revealed 70% of these to be blasts. The morphology of the blasts was not characteristic of myeloid and lymphoid lineage. Cytochemistry showed myeloperoxidase (MPO), Sudan Black B (SBB), periodic acid-Schiff (PAS) and non-specific esterase (NSE) negativity. Flowcytometry showed blasts that were positive for CD-33, CD-34 and CD-7. On second follow-up on the 10th day, the same picture persisted on morphology. On subsequent follow-up, the blasts disappeared. This was thus confirmed to be a case with transient leukemia with Downs syndrome.

Keywords: Downs syndrome, transient myeloproliferative disorder, transient leukemia

How to cite this article:
Anand H, Bakhshi S, Saxena R. Transient leukemia. Indian J Pathol Microbiol 2009;52:257-9

How to cite this URL:
Anand H, Bakhshi S, Saxena R. Transient leukemia. Indian J Pathol Microbiol [serial online] 2009 [cited 2020 Jul 5];52:257-9. Available from: http://www.ijpmonline.org/text.asp?2009/52/2/257/48938

   Introduction Top

Infants with Downs syndrome (DS) infrequently develop a condition known as transient leukemia (TL) or transient myeloproliferative disorder. It is characterized by acute leukemia and a high number of circulating blasts. We present here a female neonate born to a G2 P1 L1 by lower cesarean section to a 38-year-old mother. At birth, the baby was noticed to have a mongoloid slant, brachycephaly, low-set ears and polydactyly of the left lower limb, suggestive of DS. The baby was generally doing well. There was no cardiovascular disturbance.

   Case Report Top

The blood count at birth showed leucocytes [white blood cells (WBCs) 57 x 10 3 /無] and thrombocytopenia (90 x 10 3 /無), with hemoglobin of 16g/dL. The peripheral blood smear showed 70% blasts, 1% monocytes, 11% neutrophils, 5% band forms, 3% myelocytes, 10% lymphocytes and five nucleated red blood cells. The red blood cells showed anisocytosis, poikilocytosis and target cells. Bone marrow examination was not performed and the patient was kept on follow-up.

Pathological findings

Morphological and cytochemical studies

The May Grunwald Giemsa-stained peripheral blood smear showed numerous blasts with a high nuclear to cytoplasmic ratio [Figure 1]. Cytochemical stains including MPO, SBB, PAS and NSE were negative.

Immunophenotypic analysis by flow cytometry

Immunophenotyping was carried out on the peripheral blood using a BD FACS CANTO six color flowcytometer.

Immunophenotyping using side scatter (SSC) and leucocyte common antigen SSC/CD45 detected a dim population as the blast. Blasts were positive for CD34, CD33 and with coexpression of CD7 and negative for CD13, CD2, CD19 and CD10 [Figure 2]. On the basis of morphology and immunophenotype, the diagnosis of transient myeloproliferative disorder was given. The patient was followed closely. The clinical status was normal.

On the 20th day, an almost similar picture persisted. After the 8th week, the WBC count returned to normal (WBCs 21 x 10 3 /無), with hemoglobin 13g/dL and platelets 200 x 10 3 /無. Neutrophils 29%, lymphocytes 64%, monocytes 2%, eosinophils 4%, basophils 1% and no blasts were detected in the peripheral blood.

   Discussion Top

TL is well known in DS. TL is also known as transient myeloproliferative disorder and transient abnormal myelopoiesis. The association of DS and leukemia has been documented for over 50 years. The incidence of leukemia in DS patients is 10-20-fold higher than that in the general population. [1] A significant proportion of DS patients with transient myeloproliferative disorder (TMPD) after initial resolution may subsequently develop acute myeloid leukemia.

In TMPD, during first few days of life, blasts appear in the peripheral blood and bone marrow and there is a spontaneous resolution within a few weeks. [2]

According to Kurahashi et al , morphologically, these blasts appeared to be of the undifferentiated type. Phenotypically, the blasts were positive for CD-41, 42b, CD-61 and CD-34, with coexpression of CD-7. [3]

Transient myeloproliferative disorder with erythroid differentiation has also been reported. [4] Immunophenotype showed that the blasts were negative for CD-34, CD-13, CD-33, CD-14, HLA-DR, CD-2, CD-3, CD-4, CD-5, CD-7, CD-8, CD-10, CD-19, CD-20, CD-22, CD-41, CD-61 and CD-71. This phenotype was consistent with abnormal erythroid cells and was compatible with the morphologic findings and weak positivity with NSE.

Our case showed negative cytochemistry to MPO, PAS, NSE and SBB. On immunophenotyping, it showed positivity for CD-34, CD-33 and CD-7. CD33 61%, CD34 54%, CD7 47%, CD10 2.3%, CD13 6%, CD19 0.6%, CD 2 7% and HLA DR 2.1%.

TL should be differentiated from congenital leukemia. The incidence of acute lymphoblastic leukemia (ALL) was very less as compared with acute myeloid leukemia (AML) (21% ALL vs 64% AML). [5] Cases of TMPD are much more stable and have fewer clinical symptoms. Hence, it is important that in all neonates, especially with DS, if blasts are seen on peripheral blood smear examination one should keep the diagnosis of TMPD.

   References Top

1.Robison LL, Nesbit ME Jr, Sather HN, Level C, Shahidi N, Kennedy A, et al . Down syndrome and acute leukemia in children: A 10 year retrospective survey from Children's Cancer Study Group. J Pediatr 1984;105:235-42.  Back to cited text no. 1  [PUBMED]  
2.Nagao T, Lampkin BC, Hug GA. Neonate with Down's Syndrome and transient abnormal myelopoiesis: Serial blood and bone marrow studies. Blood 1970;36:443-7.  Back to cited text no. 2    
3.Yumura-Yagi K, Hara J, Kurahashi H, Nishiura T, Kaneyama Y, Osugi Y, Mixed phenotype of blasts in acute megakaryoblastic leukemia and transient abnormal myelopoiesis in Down's Syndrome. Br J Hematol 1992;81:520-5.  Back to cited text no. 3    
4.Bozner P. Transient myeloproliferative disorder with erythroid differentiation in Down syndrome. Arch Pathol Lab Med 2002; 126:474-7.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Ho MH, Ha SY, Chan GC, Ma ES. Leukemia or Leukamoid, Down's syndrome or not? Haematologica 2004;89:ECR 33.  Back to cited text no. 5    

Correspondence Address:
Renu Saxena
Department of Haematology, 1st Floor IRCH Building, All India Institute of Medical Sciences, Ansari Nagar, New Delih - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.48938

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