Juvenile Myelomonocytic leukemia, 3 patients HD

05.11.2013
Quality Assurance Program of Hematology Juvenile Myelo-Monocytic Leukemia ,JMML. Juvenile myelomonocytic leukemia , JMML, is a serious chronic leukemia (cancer of the blood) that affects children aged 0-14 years. The average age of patients at diagnosis is 2 years old. The World Health Organization has included JMML in the category of Myelodysplastic and Myeloproliferative disorders First Case 2 years old Male WBC: 64.000 , Hb: 10.8, plt: 162.000 PBS differential count: Blast: 2% , myelocytes: 7%, metamyelocytes: 3%stab: 16% , segmented: 48%, Lymphocytes: 5%Monocytes: 14%, Eo: 1%, basophils: 4%erythroblast: 2/100 BM asp report: Hypercellular explosive marrow. BM asp differential count:Blast: 4%, myelocytes: 18%. metamyelo: 8%, stab: 24%segmented: 27%, Lymphocytes: 10%, Monocytes: 5%, Eo: 3%, baso: 1% The Second Case 6 months old female, with Hepatosplenomegaly WBC: 55.000Hb: 8.4plt: 181.000DLC:P45 L 10 M20, promyelo 6, myelo 9, meta 7, blast 3%, Neutrophils showing dysplasia and left shift. BM asp report : Hyercellular marrow with granulocytic hyperplasia and dysplasia, increased monocytes and eosinophils.Mild dyserythropoiesis. Blast 5%. The Third Case 10 years old male,with cervical lymphadenopathy and chest nfection. WBC: 55.000 Hb: 8.4 plt: 181.000 DLC: P45 L 10 M20, promyelo 6, myelo 9, meta 7, blast 3%, Neutrophils showing dysplasia and left shift. BM asp report : Hyercellular marrow with granulocytic hyperplasia and dysplasia, increased monocytes and eosinophils. Mild dyserythropoiesis. Blast 5%. Diagnosis The following criteria are required in order to diagnose JMML: All 3 of the following: 1-No Philadelphia chromosome or BCR/ABL fusion gene. 2- Peripheral blood monocytosis more than1 000/mcL. 3-Less than 20% blasts including promonocytes in the blood and bone marrow blast count is less than 2% on average Two or more of the following criteria: 1-Hemoglobin F increased for age. 2- Immature granulocytes and nucleated red cells in the peripheral blood. 3-White blood cell count more than10.000/mcL . 4-Clonal chromosomal abnormality e.g., monosomy 7. 5-Granulocyte macrophage colony-stimulating factor GM-CSF, hypersensitivity of myeloid progenitors in vitro. The differential diagnosis list includes infectious diseases like Epstein-Barr virus, cytomegalovirus, human herpesvirus 6, histoplasma, mycobacteria, and toxoplasma, which can produce similar symptoms. These cases were studied by his excellency Dr. Ali bin Saad Alshammary , general director of laboratories and blood banks, and Dr. Mohamad Khair Ahmad

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