MYELODYSPLASTIC AND MYELOPROLIFERATIVE

Transcription

1 MYELODYSPLASTIC AND MYELOPROLIFERATIVE DISORDERS Pediatric Hemato-Oncology Division Medical Faculty University of Sumatera Utara 1

2 MYELODYSPLASIA SYNDROME A group of disorder defect in hematopoetic cell development Progresses from dysplastic ineffective hematopoesis to aggressive overt myelogenous leukemia 2

3 These are classified as : Refractory anemia (RA) Ra with ringed sideroblasts Ra with excess blasts Ra with excess blasts in transformation Chronic myelomonocytic leukemia (CML) Close relationship to aml patients treated with AML protocols 3

4 NORMAL BONE MARROW SMEAR, MAY-GIEMSA STAIN, X100 TAKEN FROM: bcl.med.harvard.edu/.../proj/raspap/bm-rars.jpg 4

5 400X MAGNIFICATION OF A BONE MARROW WITH PRIMARY REFRACTORY ANEMIA TAKEN FROM: 5

6 REFRACTORY ANEMIA WITH RINGED SIDEROBLASTS (RARS) BONE MARROW SMEAR, IRON STAIN, X1000 TAKEN FROM: bcl.med.harvard.edu/.../proj/raspap/bm-rars.jpg 6

7 REFRACTORY ANEMIA WITH EXCESS OF BLASTS, BONE MARROW SMEAR TAKEN FROM: citylightsnews.com/.../raeb-excess_blasts.jpeg 7

8 REFRACTORY ANEMIA WITH EXCESS OF BLASTS IN TRANSFORMATION (RAEB-T) BONE MARROW SMEAR, MAY-GIEMSA STAIN, X200 TAKEN FROM: bcl.med.harvard.edu/.../proj/raspap/bm-rars.jpg 8

9 CHRONIC MYELOMONOCYTIC LEUKEMIA PERIPHERAL BLOOD TAKEN FROM: 9

10 ACUTE MYELOMONOCYTIC LEUKEMIA - MICROSCOPIC View TAKEN FROM:medicalimages.allrefer.com/large/acute-myelom. 10

11 These disorder are characterized by a single lineage myeloid proliferation that can progress to AML-like leukemia, include : CML Essential thrombocytopenia (ET) Policythemia Vera (PV) Agnogenic myeloid metaplasia Juvenile myelomonocytic leukemia 11

12 Treatment : Conservative management If failed or diseases progress to AML-like leukemic consider Stem cell transplantation 12

13 THROMBOCYTOSIS Thrombocyte count above the normal value for age Varies between platelet counts of > > 1000 x 10 9 /l Thrombocytosis are classified as : Mild : > < 700 x 10 9 / l Moderate : x 10 9 / l Severe : > 900 x 10 9 / l Extreme : > 1000 x 10 9 / l 13

14 Cause of an increase in platelet count : 1. A primary disorder, such as myelo-proliferative or dysplastic syndrome ( An essential or primary thrombocytosis) 2. Increased production due to stimuli 3. A shift in platelets from the splenic pool into the peripheral circulation Reactive or secondary thrombocytosis 14

15 Table 1. differences between essential and reactive thrombocytosis Age ( years) Duration Origin Microvascular symptoms Thrombosis Bleeding Splenomegaly Platelet count ( x 10 9 /l ) Platelet morphology Platelet function Platelet distribution Iron stores Acute phase reagents such as IL-6, CRP, fibrinogen Essential (primary) Mostly > 20, often > 40 Over 2 years Stem cell defect Often Often Often Often Mostly > 1000 Large, dysmorphic Disturbed Elevated Normel Normal Reactive (secondary) Mostly < 20 Days or weeks, sometimes months Reaction to hypoxemia, infection, platelet loss; shift of platelet pool Extremely rare Extremely rare Extremely rare Rare Mostly < 1000 Large, normal appearance Normal Normal width Low High, if thrombocytosis caused by infection 15

16 ESSENTIAL THROMBOCYTOSIS Rare in children and young or middle-aged adults Most common in the fifth or sixth decade d of life Clinical i l manifestation ti persistent elevated platelet count > 1000x10 9 /l Splenomegali li Recurrent bleeding Microcirculatory disturbances : acrocyanosis, myocard infarction, transient ischemic attack (TIA) asypmtomatic 16

17 Treatment Platelet lowering therapy ( e.g. hydoxyurea, busulfan, anagrelide, interferon, radioactive phosphorous, platelet apheresis) Platelet aggregation inhibition ( e.g. aspirin and dipyridamole) 17

18 REACTIVE THROMBOCYTOSIS Pathophysiology Stimulation of thrombocyte production after peripheral loss of thrombocyte ( e.g. after immunologic, septic, oncogenic or traumatic events, blood loss or hypoxemia, of respiratory or cardiac origin ) Shift of pool into the peripheral blood (exercise, stress, inj. Epinephrine p and isoprenaline, asplenia) 18

19 Incidence More common than essential thrombocytosis Higher incidence in neonates, infants, and young children Incidence between 6-13% in hospitalized children, and 15% in pediatric outpatients = 78% mild thrombocytosis 15% moderate thrombocytosis 7% severe thrombocytosis 19

20 Table 2. Conditions associated with reactive thrombocytosis ( predominantly in infants and young children) Infection Respiratory Meningitis iti Gastrointestinal Tissue damage ( surgery, trauma) Splenectomy Hypoxemia Anemia Iron-deficiency anemia hemolytic anemia Anemia due to blood loss Anemia caused by nephrotic disease Respiratory disease Cardiac hypoxemia 20

21 Table 2. Conditions associated with reactive thrombocytosis ( predominantly in infants and young children) Autoimmune disease Juvenile rheumatoid arthritis Kawasaki syndrome Henoch-schoenlein disease Renal disease Malignancy hepatoblastoma Hodgkin s disease Histiocytosis Sarcoma Acute lymphoblastic leukemia and Non-hodgkin lymphoma Prematurity 21

22 Table 2. Conditions associated with reactive thrombocytosis ( predominantly in infants and young children) Stress situation Medication Epinephrine Corticosteroid Vinca alkaloids l Miconazole Penicillamine Methadone ( during pregnancy) Hydantoin ( during pregnancy ) Miscellaneous gastroesophegeal reflux cafley s disease 22

23 Complications Thrombosis Headache Confusion Convulsions Cerebral Infarction Intracranial hemorrhage Hemiparesis 23

24 Indications for prophylaxis Prophylaxis with anticoagulants or platelet aggregation inhibitors if risk factors exist : Immobilization in a cast Leukemia Alterations ti of other plasmatic thrombophilic hili factors 24

25 Indications for prophylaxis Iron-deficiency anemia Cyanotic heart disease Cardiac arrythmias after fontan surgery Splenectomy for a myeloproliferative syndrome or hematologic disease Post operative thrombocytosis after pancreas transplantation 25

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