Evaluation Of Serum Hepcidin Concentration In Children With Acute Leukemia:

Sheren Elsayed Shaheen ;

Acute leukemia is a malignant disease which is characterized by disordered differentiation of eitherlymphopoietic stem cells (acute lymphoblastic leukemia),or hemopoietic stem cells (acute myeloid leukemia) .The leukemias are the most common malignantneoplasms in childhood, accounting for about 41% of allmalignancies that occur in children <15 year of age.Children with acute leukemia generally present withsigns and symptoms that reflect bone marrow infiltrationand extramedullary disease. Because the bone marrow isreplaced with leukemic blasts, patients present with signsof bone marrow failure, including anemia,thrombocytopenia, and neutropenia. Clinically, themanifestations include fatigue and pallor, petechiae andbleeding, and fever .Hepcidin, predominantly produced in the liver, hasturned out to be a key iron regulator in humans by bindingto ferroportin, the only known cellular iron exporter invertebrates, and causes its internalization, thus decreasingiron efflux from iron exporting tissues into plasma.Hepcidin expression is up-regulated by iron; by cytokines(IL-1 and IL-6); and by the bone morphogenetic proteins(BMPs). It is down-regulated by anemia and hypoxia.Dysregulation of hepcidin or its receptor ferroportinresults in a spectrum of iron disorders. In inflammatorydisorders and infections, cytokine-induced hepcidin excesscontributes to development of anemia of inflammation,characterized by hypoferremia and anemia despite adequateiron stores.A normocytic, normochromic anemia is common inpatients with a variety of inflammatory disorders, includingmalignancy.Generally, the chronic anemia associated withcancer is characterized by an inadequate production of Epofor a given hemoglobin/hematocrit as well as an inadequateresponse of the erythroid marrow to endogenous Epo. Inaddition, there is impaired release of iron from stores as aresult of increased hepcidin production.This study was carried out on 25 children with acuteleukemia admitted to national cancer institute in cairo.,fifteen males and ten females. Their ages ranged from 1-15years. Ten children of matched age and sex were taken as areference group.Patients and reference groups were subjected to detailedhistory taking and thorough clinical examination : speciallyfor pallor, fever, bone ache, hepatosplenomegaly andlymphadenopathyRoutine laboratory assessment was done measuringCBC, bone marrow examination ,Immunophenotyping ofmarrow samples,LDH, ESR, serum iron and ferritin.Specific investigations were done measuring serumhepcidin with ELISA.The summary of the results were as the following :The majority (16) of patients (64%) aged between 2-10years with male to female ratio 1.2:1.Manifestations ofbone marrow failure in the form of pallor and bleedingtendancy represent the most common initial clinicalpresentations followed by manifestations ofextramedullary involvement in the form ofhepatoslenomegaly .Regarding the FAB morphologicalclassification L2 morphology was the most commonsubtype 55% of cases.As regared immunophentyping(75%) of cases were B-lineage and (25%) were Tlineage,precursor-B was present in70% of the totalALL cases. As regard FAB classification of AMLpatients more than 70% of cases was M2 and M3.Thestudy of complete blood picture of the patients showsSummary &conclusion106leukocytosis was present in more than one half ofpatients with ALL with initial leukocytic count greaterthan 50000/mm2 is present in approximatly 20% ofpatients and (Hb < 10 gm/dl) excists in appoximatly80% of patients at diagnosis while thrombocytopeniaoccurs in approximatly 75%. of their patient.Serumferritin increase while serum iron decrease in pediatricpatients with acute leukemia .Serum hepcidin inpediatrics patients with acute leukemia was elevated.There was significant positive correlation betweenserum hepcidin and ferritin. There was significantnegative correlation between serum hepcidin andhemoglobin . There was significant negative correlationbetween serum hepcidin and iron .We concluded that: Elevated serum hepcidin in pediatrics patients withacute leukemia may lead to functional irondeficiency which may play a role in the etiology ofanemia in cancer patients. The role of hepcidin in explanation of anemiaspecially of hematological malignancy is not clear. Hepcidin is one of contributing factors of anemia inacute leukemia.