title: Adult acute lymphoblastic leukemia with near haploidy, hyperdiploidy and Ph positive lines: a rare entity with poor prognosis.
abstract: Chromosomal ploidies provide a wealth of information with respect to the prognosis of patients with acute lymphoblastic leukemia (ALL). The prognosis is favourable in hyperdiploidy (>50 ALL), whereas pseudodiploidy and hypodiploidy have an overall poor clinical outcome. Near haploid ALL with less than 30 chromosomes in the leukemic blasts has been reported. This is an extremely rare malignancy with an adverse clinical course compared to other lymphoblastic leukemias. We present a case of near haploid ALL in an adult male with a diagnosis of pre-B-cell ALL. The presenting features and relevance to the progression of disease are discussed with respect to the near haploid lines. Occurrence in an adult male and the presence of additional clones with structural abnormalities are both unique to the present study. Caution must be exercised when the hyperdiploid metaphases consists exclusively of tetrasomic chromosomes and morphological examination reveals definite dual populations of large and small lymphoblasts. Despite current developments in ALL therapy, near haploid ALL continues to be a disease with an adverse clinical outcome and newer therapeutic strategies are warranted for better management of the disease.
authors.affiliation: Divisions of Cancer Research, Regional Cancer Centre, Medical College Campus, Trivandrum, Kerala, India.
authors.forename: Sidharthan K
authors.initials: SK
authors.lastname: Sunil
authors.forename: Purushothaman N
authors.initials: PN
authors.lastname: Prakash
authors.forename: Sreedharan
authors.initials: S
authors.lastname: Hariharan
authors.forename: Gopalakrishnan
authors.initials: G
authors.lastname: Vinod
authors.forename: Ramadas T
authors.initials: RT
authors.lastname: Preethi
authors.forename: Narayanan
authors.initials: N
authors.lastname: Geetha
authors.forename: Ravindran
authors.initials: R
authors.lastname: Ankathil
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D020732:Cytogenetic Analysis; D017809:Fatal Outcome; D006238:Haploidy; D006801:Humans; D008297:Male; D010677:Philadelphia Chromosome; D011003:Ploidies; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D011379:Prognosis; D012008:Recurrence; D017211:Treatment Failure