A four year old boy was admitted with a history of abdominal pain and fever for two months, maculopapular rash for ten days, and dry cough, dyspnea and wheezing for three days. On examination, liver and spleen were enlarged 4 cm and 3 cm respectively below the costal margins. His hemoglobin was 10.0 g/dl, platelet count 37 x 109/L and total leukocyte count 70 x 109/L, which included 80% eosinophils. Bone marrow examination revealed a cellular marrow comprising 45% blasts and 34% eosinophils and eosinophilic precursors. The blasts stained negative for myeloperoxidase and nonspecific esterase and were positive for CD19, CDIO, CD22 and CD20. Which one of the following statements in not true about this disease?
Answer is D (Inv (16) is often detected in the blasts and the eosinophils): This is a case of ALL with hypereosinophillic syndrome. Inv (16) is associated with AML and not ALL, and therefore represents the incorrect statement amongst the option Abstract Title: Granular acute lymphoblastic leukemia with hypereosinophilic syndrome. Journal Source: Ann Hematol. 2000 May;79(5):272-4. Authors: Jain P, Kumar R, Gujral S, Kumar A, Singh A, Jain Y, Dubey S, Anand M, Arya LS. A four-year-old boy presented with marked peripheral blood eosinophilia (absolute eosinophil count of 54 x 10(9)/1), features of hypereosinophilic syndrome, and acute lymphoblastic leukemia (ALL-L2), the latter characterized by the presence of granular blasts. Blasts were negative for myeloperoxidase, non-specific esterase, acid phosphatase, periodic-acid Schiff stain, and toluidine blue. They exhibited an early pre-B immunophenotype (TdT, CD19, CDIO, CD20 and CD22 positive) and stained negative for T (CD7, CD2, CD5 and CD3) and myeloid markers (MPO, CD33 and CD13). Chromosomal analysis revealed a normal karyotype. To the best of our knowledge, this case represents the first repo of the coexistence of granular ALL and hypereosinophilic syndrome. Why Hypereosinophillic Syndrome Option A Explanation Idiopathic hypereosinophillic syndrome is a rare condition characterized by extremely high levels of peripheral blood eosinophil counts. (80% of (70 x 109) Leucocytes were eosinophils in this patient) Clinical picture of dry cough, dyspnea, wheezing etc is fuher suggestive of HES. This is differentiated from eosinophillic leukaemia by the absence of eosinophilic blast cells. (Text from case repo: for option A) Explanations to options: Text from case repo 'In contrast to myeloproliftrative disorders where Eosinophils are pa of the Neoplastic clone, eosinophilia in patients with ALL is believed to be a reactive process.' - Case repo 'A normal karyotype has been observed in 45% of patients of ALL/Eo and t (5:14) rearrangement has been documented in same of them.' - Case repo `The child was treated with MCP-841 protocol of NC2 (prednisolone, L-asparginine, vincristine and daunaribicin) and was in remission after 4 weeks of chemotherapy. However, the peripheral blood eosinophils and the pulmonary symptoms normalized 6 weeks after attaining remission. - Case repo.
Eosinophils are not pa of the Neoplastic clone
t(5:14) rearrangement may be detected in blasts
Peripheral blood eosinophilia may normalize with chemotherapy
Inv (16) is often detected in the blasts and the eosinophils.