Abstract
Anaplastic lymphoma kinase (ALK) with positive Anaplastic Large Cell Lymphoma (ALCL) is has a distinct entity among the peripheral T-cell lymphomas. With a 5-year survival rate of 70%, it carries one of the best prognoses among peripheral T cell lymphomas. In rare instances, ALK positive ALCL presents in leukemic phase and it has a very poor prognosis with negligible number of cases reporting a survival rate of beyond one year. Following Case study as a research technique, the researchers tried to discuss about a patient affected by ALK positive ALCL in leukemic phase, associated with t (2;5)(p23;q35). The patient reported the clinic with a three week history of breathlessness, fever, diarrhoea, and axillary lymphadenopathy. His condition deteriorated in spite of rapid initiation of chemotherapy, and he succumbed to the high tumor burden. The study concludes that early diagnosis and institution of treatment is important in this regard. As this is a rare medical condition, with negligible alternative solutions, the present study underlines the need for further research in this area, emphasizing the need for newer therapies. It particularly stresses the need to examine the association of EBV and ALCL, with a specific reference to ALK-negative ALCL.
Highlights
Anaplastic large cell lymphoma (ALCL) is a relatively rare type of neoplasm, comprising 3% of the adult onset Non-Hodgkin Lymphoma (NHL) and 10-15% of pediatric NHL cases
We report an unusual case of a patient with ALKpositive ALCL presenting in leukemic phase, who had a history of infectious mononucleosis
Leukemic presentation of Anaplastic lymphoma kinase (ALK) positive ALCL is a rare entity among peripheral T cell lymphomas
Summary
Anaplastic large cell lymphoma (ALCL) is a relatively rare type of neoplasm, comprising 3% of the adult onset Non-Hodgkin Lymphoma (NHL) and 10-15% of pediatric NHL cases. ALK positive ALCL tends to affect children and young adults with higher male predilection It carries a good prognosis with five year survival rate of more than 80% even in advanced stage [4]. He was started on hydroxyl urea and allopurinol and transferred to our facility His blood counts on arrival to our center showed White cell count 97 K/uL, hemoglobin 12.7 g/dL, platelets 128 K/uL, serum lactate 10 mg/dL and Beta-2 microglobulin 12 mg/dl. IHC stains demonstrated that the tumor cells were positive for CD30 and ALK and were distributed in a sinusoidal pattern in the bone marrow (Figure 2). We hope that newer therapeutic agents will improve the remission and overall survival rates
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