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MPN • 1- What is Philadelphia chromosome , Describe its role in the pathogenesis of CML? • 2- Mention the phases of CML in details supported by the laboratory findings? • 3- What is the major and minor criteria used to diagnose PV by WHO • 4- What is the lab findings of essential thrombocythemia? • 5- Describe the pathogenesis of chronic idiopathic myelofibrosis? • 6-What are the peripheral blood findings of PMF? PCM and MDS · 1- What is plasma cell myeloma (MM), Enumerate the positive data that diagnose it? • 2- Mention the diagnostic criteria of smouldering PCM • 3- What is the definition of MDS, Mention the WHO classification supported by peripheral blood and bone marrow findings. • 4- Describe the morphological abnormalities in peripheral blood and bone marrow smear found in Refractory cytopenia with multilineage dysplasia? • 5- Write short note on lab. Diagnosis of CMML.

Answer 1

MPN

   1) Philadelphia chromosome

Philadelphia chromosome is the first tumour specific chromosomal change. It is a shortened chromosome 22 resulting from reciprocal translocation of part of chromosomes 22 and 9.It is found only in the affected blood cells. Because of the damage to DNA the chromosome produce abnormal enzyme called Tyrosine kinase.

Role in Pathogenesis of CML

   Philadelphia chromosome affect multiple signaling pathway that directly affect apoptotic Potential, cell division rates, and different stage of cell cycle to achieve unchecked proliferation characteristics of CML and ALL(acute lymphoblastic leukemia).

2)Phases of Philadelphia chromosome

Chronic phase

Accelarated phase

Blast phase

PHASE	Chronic phase	Accelerated phase	Blast phase
blood sample	less than 10%blast in their blood	less than 305blast in their blood	20%or more blast
symtoms	Fairly mild symptom	fever, poor appetite, weight loss	fever, poor appetite, weight loss.

3)Major and minor criteria used to diagnose PV by WHO

Major criteria	Minor criteria
Haemoglobin >16.5g/dl in men,.16.0 in women Presence of JAK2V617Fand JAK2 exon 12	Serum erythropoietin

4) Most people are without symptoms, at the time of diagnosis, which is usually made after noting an elevated platelets level on a routine complete blood count.

5)

6)The peripheral blood findings are,

   immature WBC'S

   immature RBC'S

abnormal RBC's morphology including anisocytosis and poikilocytosis

PCM and MDS

2) Serum monoclonal protein>or=30g/L

   urinary monoclonal protein>=500mg per 24 h

   clonal bone marrow plasma cells10-60%