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38 Cards in this Set
- Front
- Back
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MPD Concepts:
... haematopoeitic disorders ... of one of myeloid lineages -Granulocytic -Erythroid -Megakaryocytic Relatively normal maturation Bone marrow ... in all MPD -secondary event Acute Myeloid Leukemia may occur |
Clonal
Proliferation fibrosis |
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MPD Concepts:
Normal (effective) maturation -... number of red cells, granulocytes, platelets (Note: myeloproliferation in MDS is ineffective) -Frequent overlap of the clinical, laboratory & morphologic findings *Leucocytosis, thrombocytosis, increased megakaeryocytes, fibrosis & organomegaly blurs the boundaries ... -Sequestration of excess blood -Extramedullary haematopoiesis -Leukaemic infiltration |
Increased
Hepatosplenomegaly |
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look at slide 8
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Incidence and Epidemiology:
Disease of ... Peak incidence in 7th decade Incidence: 6-9/100,000 |
adult
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Abnormalities of ... in MPD’s:
PRV >90% ET 40% IMF 50% |
Jak-2
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...:
Definition -Raised packed cell volume (PCV / HCT) -Male > 0.51 (50%) -Female > 0.48 (48%) |
Polycythemia
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Polycythemia Classification:
True / Absolute -Primary Polycythemia (...) -Secondary Polycythemia *... dependent .Hypoxia dependent .Hypoxia independent *... independent Apparent / Relative -Reduction in plasma volume |
PRV
Erythropoietin Erythropoietin |
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... is the main protein that leads to the formation of hemoglobin.
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erythropoietin
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look at slide 15
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Causes of secondary polycythemia:
EPO RECEPTOR–MEDIATED -Activating mutation of the erythropoietin ... DRUG-ASSOCIATED -EPO Doping -Treatment with ... Preparations |
receptor
Androgen |
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Secondary polycythemia
physiologically appropriate: -... altitude - COPD - Right to left cardiac shunt - ... syndrome (massive obesity) - High oxygen affinity hemoglobinopathy - Smokers polycythemia |
High
Pickwickian |
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Secondary polycythemia
physiologically inappropriate: ... -renal carcinoma -cerebellar hemangioblastoma -hepatoma -uterine fibroid -adrenal cortical adenoma (and/or hyperplasia) -ovarian carcinoma ... -cysts -hydronephrosis -Bartter’s syndrome -transplantation |
Tumor
Renal |
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Relative polycythaemia:
Apparent polycythaemia or pseudopolycythaemia due to ... volume contraction Causes -Stress -Cigarette smoker or ... intake -Dehydration -Plasma loss- burn injury |
plasma
alcohol |
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...:
Chronic, clonal myeloproliferative disorder characterized by an absolute increase in number of RBCs Incidence: 2-3 / 100,000 Median age at presentation: 55-60 Male/Female: 0.8:1.2 |
Polycythemia Rubra Vera (PRV)
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Polycythemia (Rubra) Vera
(PRV): Neoplastic stem cell disorder possessing a ... mutation (first proposed in 2004) Single nucleotide JAK2 somatic mutation (JAK2V617F mutation) in the majority of PV patients Excessive production of all myeloid cell lines, but predominantly ... cells |
JAK-2
red |
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PRV Disease phase:
Proliferative phase “Spent” post-polycythaemic phase -... bone marrow biopsy reticulin or collagen -... spleen size -Progressive ... -Leukoerythroblastic blood picture Rarely transformed into acute leukemia |
Increased
Increasing anemia |
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Clinical Features:
Age -55-60 years -May occur in young adults and rare in childhood Majority patients present due to ... complications -Thrombosis (including portal and splenic vein) -DVT -Hypertension -Headache, poor vision and dizziness -Skin complications (pruritus, erythromelalgia) -Haemorrhage (GIT) due to platelet defect |
vascular
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look at slide 26
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Laboratory:
Persistent leukocytosis Persistent thrombocytosis ... – don’t correct this iron deficiency! Elevated -leukocyte alkaline phosphatase -B12 -B12-binding capacity -uric acid |
Microcytosis
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Laboratory features and morphology:
Hb, PCV (HCT), and Red cell mass ... ... serum erythropoietin ... bone marrow |
increased
Low Hypercellular |
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PRV – diagnosis:
Red Cell Mass if necessary Exclude ... causes of polycythemia (measure erythropoietin) Look for features of primary polycythemia ... mutation analysis |
secondary
JAK-2 |
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look at slide 31
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Treatment of PV:
... -Target hct < 45 in men and 42 in women ... in high-risk pts for thrombosis -Age over 60 -Prior thrombosis -Platelet >1,500,000/microL -Presence of ... risk factors Aspirin (75-100 mg/d) if no CI IFNa for refractory pruritus, pregnancy Do not treat with ... |
Phlebotomy
Hydroxyurea cardiovascular iron |
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...:
Neoplastic stem cell disorder causing dysregulated production of large numbers of abnormal platelets Clonal myeloproliferative disease of megakaryocytic lineage Abnormal platelets aggregate in vivo, causing thrombosis Abnormal platelets also cause bleeding |
Essential Thrombocythemia
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Essential Thrombocythaemia (ET):
Persistent elevation of Plt>600 x109/l Incidence: 2.5 cases/100,000 Male/Female: 2/1 Median age at diagnosis: 60 (20% cases <40yrs) Lack of positive diagnostic criteria -ET is a diagnosis of ... -Clinical setting, blood film, bone marrow -40% ... mutation |
exclusion
JAK-2 |
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ET is a diagnosis of exclusion :
Criteria of exclusion: No evidence of Polycythaemia vera No evidence of CML No evidence of myelofibrosis No evidence of myelodysplastic syndrome No evidence of reactive thrombocytosis -Bleeding -Trauma -Post operation -Chronic ... def -Malignancy -Chronic infection -Connective tissue disorders -Post ... |
iron
splenectomy |
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Clinical Features of ET:
Vasomotor -Headache -Light-headedness -Syncope -... (burning pain of the hands or feet associated with erythema and warmth) -Transient ... disturbances (eg, amaurosis fujax, scintillating scotomata, ocular migraine) Thrombosis and Haemorrhage Transformation |
Erythromelalgia
visual |
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Diagnostic criteria for ET:
-Platelet count >600 x 109/L for at least 2 months -No cause for ... thrombocytosis -Normal RBC mass or a HCT < 48 -Megakaryocytic hyperplasia on BMBx -Presence of stainable ... in a BM aspiration -No evidence of myelofibrosis -No evidence of MDS -Absence of the ... chromosome |
reactive
iron Philadelphia |
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look at slide 42-43
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ET- Treatment:
None in ...-risk cases Anti-platelet agents (...) ... reduction treatment - hydrea |
low
aspirin Platelet |
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look at slide 45
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Myelofibrosis
Chronic idiopathic myelofibrosis: Neoplastic (clonal) ... stem cell disorder Distinguish from secondary marrow fibrosis Bone marrow failure Myeloid ... (extra-medullary hemopoiesis) |
hemopoietic
metaplasia |
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Myelofibrosis
Chronic idiopathic myelofibrosis: Progressive fibrosis of the marrow & increase ... element Agnogenic myeloid metaplasia -... erythropoiesis *Spleen *Liver Abnormal ... -Platelet derived growth factor (PDGF) -Platelet factor 4 (PF-4) |
connective tissue
Extramedullary megakaryocytes |
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MF- Pathology:
Connective tissue within the bone marrow --> ... deposit --> New ... formation --> Destruction of normal marrow microenvironment Circ stem cells: cells normally present in the marrow --> Dysplastic Feature ... haemopoiesis - eg. liver. |
Collagen
bone Extramedullary |
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MF – Clinical Presentation:
... onset in older people Massive ... Hypermetabolic Sx - Loss of weight, fever and night sweats Bleeding problems Bone pain Gout Marrow failure Can transform to ... in 10-20% of cases |
Insidious
Splenomegaly acute leukaemia |
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MF – Diagnosis:
Typical blood picture Splenomegaly ... aspirate ... on BM biopsy |
Dry
Fibrosis |
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look at slides 53-55
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MF – treatment:
Transfusions, ... care ... reduces splenomegaly and hypermetabolic symptoms ... for severe symptomatic splenomegaly 10%-20% transform to ... Consider allogenic-BMT |
supportive
Hydroxyurea Splenectomy acute myelogenous leukemia |
