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1 STEM CELL DISORDERS WHEREBY YOU GET ABNORMAL PROLIFERATION IN ONE OR MORE CELL LINE DERIVED FROM A COMMON STEM CELL Myeloproliferative Disorder.

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Presentation on theme: "1 STEM CELL DISORDERS WHEREBY YOU GET ABNORMAL PROLIFERATION IN ONE OR MORE CELL LINE DERIVED FROM A COMMON STEM CELL Myeloproliferative Disorder."— Presentation transcript:

1 1 STEM CELL DISORDERS WHEREBY YOU GET ABNORMAL PROLIFERATION IN ONE OR MORE CELL LINE DERIVED FROM A COMMON STEM CELL Myeloproliferative Disorder

2 2 THE INDIVIDUAL FEATURE OF THESE DISEASES RESULT FROM A: DISTURBED HAEMOPOIETIC MICROENVIRONMENT CLONAL ABNORMALITY DISTURBANCE IN HAEMOPOIETIC REGULATION.

3 3 Myeloproliferative Disorder Polycythaemia Ruba Vera Myelofibrosis Primary Thrombocytopenia Chronic Myeloid Leukaemia Myeloproliferative Disorder – unclassifiable Chronic Eosinophilic Leukaemia Chronic Myeloid Leukaemia

4 4 CMPD- COMMON FEATURES Proliferation and differention of one or more stem cell. Raised W.C.C.. HB, Platelets Organomegaly Extramedullary Haematopoiesis Clinical, Laboratory and Morphological overlap

5 5 CMPD Disease of Adults Peak Onset /100,000 Limited Geographical Based Data

6 6 PLATELETS > 600 X 10 9 /L ^ MEGAKARYOCYTES IN THE MARROW CLONAL DISORDER OF THE MULTIPOTENTIAL STEM CELL PRIMARY THROMBOCYTHAEMIA

7 7 Primary Thrombocythaemi - Pathogenesis Aetiology – Unknown Megakaryocytic hyperplasia Functionally abnormal platelets

8 8 Primary Thrombocythaemia Clinical Features Asymptomatic Vasomotor- 40% Haemorrhage – 25% Thrombosis – 20% Splenomegaly Recurrent Miscarriage

9 9 PRIMARY THROMBOCYTOSIS DIAGNOSTIC CRITERIA PLATELET COUNT > 600X10 9 /L FOR OVER 2 MONTH WITH NO CAUSE OF REACTIVE THOMBOCYTOSIS, NO EVIDENCE OF PRV, MYELOFIBROSIS, MYELODYSPLASIA AND NO PH CHROMASOME

10 10 PRIMARY THOMBOCYTOSIS DIAGNOSIS: EXCLUDE CAUSE OF REACTIVE THROMBOCYTOSIS. EG:ACUTE HAEMORRHAGE MALIGNANT DISEASE, CHRONIC INFLAMM DISORDER, ACUTE INFLAM POST-OP SPLENECTOMY EXERCISE IRON DEF.

11 11 PRIMARY THROMBOCYTHAEMIA TREATMENT MYELOSUPPRESIVE HYROXUREA ANAGRELIDE ANTI-PLATELET AGENTS INTERFERON

12 12 POLYCYTHAEMIA Absolute polycythaemia Relative polycythaemia

13 13 ABSOLUTE POLYCYTHAEMIA.PRIMARY POLYCYTHAEMIA - POLYCYTHAEMIA RUBRA VERA - ERYTHROPOIETIC RECEPTOR GENE MUTATION. 2. SECONDARY POLYCYTHAEMIA - HYPOXAEMIA PO O < 92% - RENAL DISEASE - TISSUE HYPOXIA - HIGH AFFINITY HB - TUMOURS - HEPATOMAS, FIBROIDS, CEREBELLAR - HAEMANGIOBLASTOMAS - HIGH ERYTHROPOIET PRODUCTION 3IDIOPATHIC ERYTHROCYTOSIS.

14 14 Older Age , Female > Male Vascular Complications - Arterial = Venous Cerebral + Coronary - Headache - Dizziness Due to Small Vessel Occlusion. => 30-50% - Thrombotic - Art = Venus, Sml & Lrg Vessels - Haemorrhagic Peptic Ulceration - ^ Histamine Levels Prutritis % Skin Change - Pletharic Facies, Acne Roscea, CLINICAL FEATURES OF P.R.V

15 15 CLINICAL FEATURES OF P.R.V. CONTD. ^ URIC ACID - GOUT ^ BP SPLENOMEGALY - 50% LAB * ^HB ^PCV - MALE - HB 17.5G/L, PCV > FEMALE HB15.5G/L, PCV > 0.46 ^ WCC ^PLATELETS 50% X 10 9 /L ^ B12 LEUCOCYTE ALKALINE PHOSPHATASE. MARROW - HYPERCELLUAR

16 16 ^RCM > 36ML/KG IN MALES - 32ML/KG IN FEMALES NO EVIDENCE OF A CAUSE OF SECONDARY POLYCYTHAEMIA INCLUDING ARTERIAL OXYGEN SATURATION > 92% + SPLENOMEGALY (PALPABLE) IF (-) SPLENOMEGALY PALPABLE - PLATELET > ^WCC > 12 - ^ LAP/^B12 COURSE: 15-20% - MYELOFIBROSIS 2-10% - ACUTE LEUKAEMIA RX VENESECTION REGULARILY CHEMOTHERAPY, HYDROXYREA ANTIPLATELET THERAPY DIAGNOSTIC CRITERIA OF PPP OR PRV

17 17 Investigation of Polycythaemia RED CELL MASS STUDIES AIM IS TO INVESTIGATE/EXCLUDE A CAUSE OF SECONDARY POLYCYTHAEMIA CLINICAL EVALUATION PULSE OXIMETRY RENAL - URINALYSIS + RENAL ULTRASOUND ABDOMINAL ULTRASOUND NEUTROPHIL COUNT PLATELET COUNT MARROW CYTOGENETICS MARROW CULTURE SERUM ERYTHROPOIETIN ASSAYS.

18 18 PREVENTION OF VASCULAR OCCLUSIONS DELAY MYELOFIBROTIC TRANSFORMATION MINIMIZE ACUTE LEUKAEMIC TRANSFORMATION. PHLEBOTOMY MYELOSUPPRESSIVE ANTIPLATELET AGENT. MANAGEMENT OF P.R.V

19 19 P.R.V. COURSE: 15-20% - MYELOFIBROSIS 2-10% - ACUTE LUEKAEMIA RX: VENESECTION REGULARLY CHEMOTHERAPY 35 p HYDROXYURIA ANTIPLATELET THERAPY

20 20 MYELOFIBROSIS (agnogenic myeloid metaplasia) 1 o DISORDER - OR - AS PART OF OTHER MYELOPROLIFERATIVE DISORDERS 20% HAVE HX OF PRV 2 ND LYMPHOPROLIFERATIVE, BENZENE, FLUORINE, ANSENIC

21 21 MYELOFIBROSIS (agnogenic myeloid metaplasia) PATHOLOGY: ^ Connective tissue within the bone marrow. ^ Collagen ^ New bone formation destruction of normal marrow microenvironment ^ circ stem cells: cells normally present in the marrow Dysplastic Feature. Extramedullary haemopoiesis - eg. liver.

22 22 MYELOFIBROSIS (agnogenic myeloid metaplasia) SYMPTOMS: OFTEN ASYMPTOMATIC: BONE MARROW FAILURE ^ SPLEEN - LUQ PAIN METABOLIC CONSEQUENCE OF M/P DISORDER - SWEATS ^URIC ACID GOUT, RENAL COLIC BLEEDING DIATHESIS

23 23 Myeloproliferative Disorders Chronic Granulocytic Leukaemia First malignancy associated with a recurring chromosomal abnormality Translocation of genetic material from chromosomes 9  22 Fusion gene  fusion protein - pathogenesis

24 24 CHRONIC GRANULOCYTIC LEUKAEMIA = CHRONIC MYELOID LEUKAEMIA 1 /100,000 MALE > FEMALE 5 TH - 6 TH DECIDE BUT CAN OCCUR AT ANY AGE PH CHROMOCOSME - RECIPROCAL TRANSLOCATION BETWEEN CHROMOSOME 9 => 22 = ? AETIOLOGICAL SIGNIFICANCE OR ? MARKER DISEASE. => CLONAL DISORDER OF HAEMOPOIETIC STEM CELL ? PROCESS - GROWTH ADVANTAGE => X 30 FIELD ^ IN GRANULOCTE MASS

25 25 CLINICAL FEATURES: BIPHASIC OR TIRPHASIC DISEASE CRONIC ACCELERATED TRANSFORMATION 20% ASYMPTOMATIC NON-SPECIFIC COMPLAINTS SPLENAMEGALY AND HEPATOMEGALY C.G.L.

26 26 C.G.L. LAB FEATURES: LEUCOCYTOSIS x 10 9 /L. BASOPHILIA THROMBOCYTOSIS HYPERCELLULAR MARROW PH POSITIVE IN 90% INCREASED MARROW FIBROSIS.

27 27 C.G.L. TREATMENT OF C.G.L: BONE MARROW TRANSPLANT CYTOREDUCTIVE THERAPY TYROSINE KINASE INHIBITORS E.G. HYDROXYUREA, INTERFERON MANAGEMENT OF METABOLIC COMPLICATIONS.


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