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only need 4 slides on clinical presentations of AML
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HAEMATOLOGY ONCOLOGY
kibaru
OBJECTIVES
At the end of the session the students should be
able to
Understand the common haematological
Malignancies
▪
epidemiology
▪
clinical presentation
▪
diagnosis
▪
summary of treatment
INTRODUCTION
In terms of frequency in the decending order are
Leukemia
Brain tumors
Lymphoma
Acute leukemia, the most common form of cancer
in children, comprises approximately 30 percent
of all childhood malignancies, with acute
lymphoblastic leukemia (ALL) being five times
more common than acute myeloid leukemia
(AML)
LEUKEMIA
Characterized by persistent and enoumous
production of immature white blood cells
It is responsible for majority of childhood
malignancy
About 98% are acute forms
ALL accounts for 76%
AML accounts for 20%
And CML 4%
ACUTE LYMPHOCYTIC LEUKEMIA
(ALL)
Commonest malignancy in children
ALL is classified as
Standard risk ALL
High risk ALL
T cell ALL
B cell ALL
EPIDEMIOLOGY
The
worldwide incidence is
1:25,000 in the white population
in the black race incidence is 23x less.
Kenya
should have over 250 children with
ALL per year, the majority probably not
reaching treatment centers.
The peak age In the first 5years mostly 2-5
years
The second peak occurs between 8-10years of
age
85% of the patients is diagnosed between
ages 2 and 10 years.
PATHOGENESIS
ALL
results from uncontrolled
proliferation of immature
lymphocytes.
Its
cause is unknown, genetic
factors, exposure to radiation and
chemicals may play a role.
▪Children with Down syndrome have
increased risk for developing both
ALL and acute myeloid leukemia.
▪
Increased occurrence of ALL is
associated with certain genetic
conditions, including
▪ Neurofibromatosis
▪
▪
▪
Shwachman syndrome
Bloom's syndrome
ataxia telangiectasia.
Signs
ALL
and symptoms
is a great immitator and the presentation
may be vague and varied resembling almost any
disease
The onset is acute or insidious
Initial presentation include progressive
pallor,anorexia,fever,malaise,lymphadenpathy,hep
atosplenomegally,purpura,epistaxis,black eye
Bone and joint pains,sores in the mouth
Pallor
petechiae
purpura
bone pains
hepatosplenomegaly
lymphadenopathy
CNS presentation
Leads to menigeal leukemia
It presents with headache vomiting,
drowsiness,unconsciousness convulsion and
cranial nerve palsy
Differential
diagnosis include
chronic infections as Epstein Barr
Virus (EBV) or cytomegalovirus
(CMV);
Immune thrombocytopenic purpura
(ITP)
auto immune haemolytic anemia
aplastic anaemia
juvenile rheumatoid arthritis (JRA).
The diagnosis of CNS leukemia requires one of
the following:
●Cytologic confirmation of the presence of
leukemic cells in the cerebrospinal fluid (CSF)
●Clinical signs of CNS leukemia such as facial
nerve palsy, brain/eye involvement, or
hypothalamic syndrome
●A tumor mass involving the CNS as determined
by imaging studies
INVESTIGATIONS
Serum LDH levels may
help distinguish JRA from
leukemia
CXR_ may show mediastinal widening or
anterior mediastinal mass and tracheal
compression due to lymphadenopathy or
thymic infiltration especially in T-cell ALL..
Haemogram shows
combination
of anemia
thrombocytopenia
leucopenia
or leucocytosis
Peripheral blood picture
often lymphoblasts in the peripheral
blood smear.
Indications of Bone marrow aspirate
●Atypical cells in the peripheral blood
●Unexplained depression of more than one
peripheral blood element (cytopenias).
Cytopenias are defined as an absolute neutrophil
count (ANC) of 50.000, then aim at
Hb 8 mg/dl
▪ Platelets transfusion if platelets < 10 x 109 or
if bleeding.
▪ Treat infections
Do
baseline investigations as HIV,
creatinine, electrolytes, uric acid,
ALT, BS for MP’s, stool O/C
Do
diagnostic lumbar puncture once
platelets >50x109, give IT
methotrexate and hydrocortisone.
If
necessary do LP after platelet transfusion.
Use sedation and good LP technique to prevent
leukaemic spread.
Start PO Allopurinol 10mg/Kg In TID Ideally
24 Hours Before Start Of Prednisone /
Chemotherapy
Start
PO Prednisone 60mg/M2 TID (Lower
With WBC >50.000 X 106:
Start 10mg/M2,
Increase Daily With 10 Mg/M2 Till Dose Of 60
Mg/M2 Is Reached At Day 6
Hydrate
24 hours before and after
cytotoxics, 3000 ml/m2 with
bicarbonate 40 mEq/l as long as
tumor load is high.
INDUCTION
IV Vincristine 2mg/m2 (max 2.5 mg)
IV Adriamycin 25 mg/m2 (infusion one hour)
IM L-Asparaginase 6000U/m2 (3 doses)
IT Methotrexate / Hydrocortisone
In CNS disease ** 3 drugs weekly
IT Methotrexate / Hydrocortisone and Cytosar
PO Prednisone 60mg/m2 in TID week 1,2 and 4,5
Consolidation...