Professional Documents
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Myeloproliferative
disorders
Done by: Dr. Mahmoud Ayesh
The Lecture was taken on
Tuesday 10\11\2009
Overview:
These make up a group of chronic
conditions characterized by clonal
proliferation of one or more
marrow cell linage Myelo- =
related to bone marrow.
It is important to determine if it is
clonal or not because if it isnt
clonal that means it is a reactive
process such as increasing RBC
in hypoxia, increasing WBC in
infections, and increasing platelets
in haemorrhage.
Remember that the most physical
sign in all myeloproliferative
disorders is the splenomegaly.
Clinical features:
Wt loss, lassitude, anorexia, night
sweats, Splenomegaly, Anaemia
pallor,
dyspnoea,
tachycardia
Bruising, epistaxis, menorrhagia,
Gout, renal impairment, and 25% of
patients are asymptomatic.
Case presentation:
Wt 3kgs
Normal c200gms
Page 2 of 9
Natural history:
The disease has three phases:
1) A chronic phase, most patients85%
diagnosed in chronic phase, characterized by
splenomegaly and leukocytosis with generally
few symptoms. This phase is easily controlled
by medication. The major goal of treatment
during this phase is to control symptoms and
complications
resulting
from
anemia,
thrombocytopenia,
leukocytosis,
and
splenomegaly. Newer forms of therapy aim at
delaying the onset of the accelerated or blastic
phase, duration3-4 years.
2)
An accelerated phase,
Characterized by
the emergence of a discrete blast cell population
and left-shift compared to chronic phase. But
the blast still less than 20%.
3)
Blast phase,
Management
1. Curative treatment: by allogeneic
2.
Page 3 of 9
Clinical features:
In this disease the platelets become as a
clusters which leads to thrombosis, so
the patient come with erythromelagia
painful big toe, CVA such as TIA or
stroke, or he may come with bleeding.
Management
Aspirin useful to reduce the risk of
thrombosis, also we can use hydroxyurea.
Clinical features
The increase in whole blood
viscosity causes vascular occlusion
and ischemia, compounded by the
increase in platelets.
Headaches, Itch, Thrombosis, TIA,
stroke, and Splenomegaly are also
finding.
Diagnosis
1) The most important thing is to
distinguish it from secondary
PRV, e.g.
2)
4)
5)
6)
Page 4 of 9
18.0
200
0.62
75
850
[4-11]
[140-180]
[.42-.51]
[80-100]
[150-450]
Neuts x 109/L
Lymphs x 109/L
Monos x 109/L
Eos x 109/L
Basos x 109/L
14.6
2.0
0.8
0.1
0.5
[2-7.5]
[1.5-4]
[0.2-0.8]
[0-0.7]
[0-0.1]
4. Myelofibrosis (MF)
Is characterized by bone marrow
fibrosis, extramedullay haematopoiesis
blood cell formation outside the bone marrow and
a leukoerythroblastic blood picture.
[4-11]
[140-180]
[80-100]
60[150-450]
[2-7.5]
1.0
[1.5-4]
0.2
[0.2-0.8]
0.1
[0-0.7]
0.1
[0-0.1]
Blood film:
A few nucleated red cells,
myelocytes (leukoerythroblastic).
Tear-drop poikilocytes.
[4-11]
[140-180]
[80-100]
[150-450]
[2-7.5]
[1.5-4]
[0.2-0.8]
[0-0.7]
[0-0.1]
Case2:
63 smoker male patient presented to a
clinic complaining of headache, & this
is a CBC for him:
WBC x 109/L
Hb g/L
HCt
MCV fl
Platelets x 109/L
Neuts x 109/L
Lymphs x 109/L
Monos x 109/L
Eos x 109/L
Basos x 109/L
Page 5 of 9
18.0
200
0.62
75
850
14.6
2.0
0.8
0.1
0.5
[4-11]
[140-180]
[.42-.51]
[80-100]
[150-450]
[2-7.5]
[1.5-4]
[0.2-0.8]
[0-0.7]
[0-0.1]
Case 3:
70 year male patient presented to you
has massive splenomegaly, history of
pallor with malaise..this is a CBC
for him:
WBC x 109/L
Hb g/L
MCV fl
Platelets x 109/L
Neuts x 109/L
Lymphs x 109/L
Monos x 109/L
Eos x 109/L
Basos x 109/L
2.4
88
85
60
1.0
1.0
0.2
0.1
0.1
[4-11]
[140-180]
[80-100]
[150-450]
[2-7.5]
[1.5-4]
[0.2-0.8]
[0-0.7]
[0-0.1]
Page 6 of 9
Case 4:
About chronic myeloid leukaemia
these pictures from web path, because there is no
picture about this in slide.
Remember:
Increased WBCs
Normal or high RBCs
Increased platlets
With basophilia & eosinophilia
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On blood film: all stages of maturations from blast to
neutrophil & majority of cells are myelocytes & neutrophils.
The end
Done by: Hashem Yassin
www.shifa2006.com
On facebook:
2012 J2009
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