MYELOID NEO - Med2

MYELOID NEOPLASMS
DR. MARY GAY BULIYAT,

M.D.
WHO Classification of Myeloid Neoplasms
and Acute Leukemia
• MYELOPROLIFERATIVE NEOPLASMS (MPN)
– CHRONIC MYELOID LEUKEMIA (CML)
– POLYCYTHEMIA VERA (PV)
– PRIMARY MYELOFIBROSIS (PMF)
– ESSENTIAL THROMBOCYTEMIA (ET)
• Myeloid neoplasms with eosinophilia and
rearrangement of PDGFRA, PDGFRB or FGFR1,
or with PCM1-JAK2
WHO Classification of Myeloid Neoplasms
and Acute Leukemia
• MYELODYSPLASTIC/ MYELOPROLIFERATIVE
NEOPLASMS (MDS/ MPN)
• MYELODYSPLASTIC SYNDROMES (MDS)
• ACUTE MYELOID LEUKEMIA (AML) and related
neoplasms
• Blastic plasmacytoid dendritic cell neoplasms
ACUTE MYELOID LEUKEMIA (AML)
ACUTE MYELOID LEUKEMIA
(AML)
• INFILTRATION OF THE BLOOD, BONE
MARROW, and OTHER TISSUES BY
PROLIFERATIVE, CLONAL
UNDIFFERENTIATED HEMATOPOIETIC
CELLS
• ANNUAL INCIDENCE: ~3.5/ 100,000
(U.S.)
• MEDIAN AGE AT DIAGNOSIS: 67 years
• MEN > WOMEN (4.5 vs 3.1)
(AML)
• ETIOLOGY
– HEREDITY
– RADIATION
– CHEMICAL (industrial exposure)
– DRUGS
(AML)
• ETIOLOGY: HEREDITY
– TRISOMY 21 in DOWN SYNDROME
• ASSOCIATED WITH INCREASED INCIDENCE
– DEFECTIVE DNA REPAIR
• FANCONI ANEMIA
• BLOOM SYNDROME
• ATAXIA- TELANGIECTASIA
– CONGENITAL NEUTROPENIA
• KOSTMAN SYNDROME
(AML)
• ETIOLOGY: RADIATION
– HIGH DOSE RADIATION
• ATOMIC BOMBS in Japan
• NUCLEAR REACTOR ACCIDENTS
– RISK PEAKS 5-7 YEARS AFTER EXPOSURE
– RADIOTHERAPY
• LITTLE RISK OF AML BUT CAN INCREASE WITH
EXPOSURE TO ALKYLATING AGENTS
(AML)
• ETIOLOGY: CHEMICALS
– BENZENE
• Chemical, rubber and plastic industries
– SMOKING
– Petroleum products, paint, embalming fluids,
ethylene oxide, HERBICIDES and PESTICIDES
(AML)
• ETIOLOGY: DRUGS
– CHEMOTHERAPY
• ALKYLATING AGENTS
– 4-6 YEARS AFTER EXPOSURE
– ABERRATIONS IN CHROMOSOMES 5 and 7
• TOPOISOMERASE II INHIBITORS
– 1-3 YEARS AFTER EXPOSURE
– ABBERATIONS IN CHROMOSOME 11q23
– Chloramphenicol, phenylbutazone, chloroquine,
methoxypsoralen
• Bone marrow failure
(AML)
• CLASSIFICATION
– WORLD HEALTH ORGANIZATION
• BIOLOGICALLY DISTINCT GROUPS
– CLINICAL FEATURES
– CYTOGENETIC ABNORMALITIES
– MOLECULAR ABNORMALITIES
– MORPHOLOGY
• Diagnostic cut off: 20% blasts or <20% if WITH certain
specific chromosomal rearrangements
(AML)
• WHO Classification
– IMMUNOPHENOTYPING
• DISTINGUISHES AML FROM OTHER LEUKEMIAS (ALL)
• AML with minimal differentiation
– CD 13 AND CD 117: MYELOID SPECIFIC CDs
• ACUTE MEGAKARYOBLASTIC LEUKEMIA
– CD41 AND CD61: PLATELET- SPECIFIC ANTIGENS
– CLINICAL FEATURES
• SUBDIVIDING FACTOR for AML
– THERAPY- REALTED AML; AML with related MDS features
(AML)
• GENETIC FINDINGS in AML
– t(15;17)(q22;q12) rearrangement
• Results in fusion gene PML- RARA
• DIAGNOSTIC FOR ACUTE PROMYELOCYTIC LEUKEMIA
– t(8;21) and t(15;17)
• Associated with YOUNGER AGE
– del(5q) and del(7q)
• Associated with OLDER AGE
(AML)
PROGNOSTIC FACTORS
• CHROMOSOME FINDINGS
• MOST IMPORTANT INDEPENDENT PROGNOSTIC
FACTOR
• t(15;17): VERY GOOD PROGNOSIS (~85% cured)
• t(8;21) and inv(16): GOOD PROGNOSIS (~55% cured)
• NO cytogenetic abnormality: intermediate outcome risk
(~40% cured)
• Complex Karyotype, t(6;9), OR -7: VERY POOR
PROGNOSIS
(AML)
PROGNOSTIC FACTORS
• POOR PROGNOSTIC FACTORS:
– ADVANCING AGE
– PROLONGED CLINICAL INTERVAL BEFORE DIAGNOSIS
– ANTECEDENT HEMATOLOGIC DISORDERS
– CYTOPENIAS >3 mos BEFORE DIAGNOSIS
– PRIOR USE OF CYTOTOXIC AGENTS for prior
malignancies
– LOW PERFORMANCE STATUS
(AML)
PROGNOSTIC FACTORS
• Achievement of COMPLETE RESPONSE
– BETTER OUTCOME and LONGER SURVIVAL
– PBS and Bone Marrow
– Peripheral Neutrophil count > 1000/uL AND Platelet
count > 100,000/uL
– Circulating BLAST should be ABSENT
– BONE MARROW: <5% BLAST and NO Auer Rods
– NO extramedullary leukemia
(AML)
• CLINICAL FEATURES: SYMPTOMS
– 50% of patients are symptomatic < 3 mos before
diagnosis
– Non- specific symptoms
• Anemia, leukocytosis or leukopenia, or
thrombocytopenia
– FATIGUE: first symptom in 50% of patients
– FATIGUE and WEAKNESS: most common complaint
at diagnosis
(AML)
• CLINICAL FEATURES
– OTHER SYMPTOMS
• ANOREXIA and WEIGHT LOSS
• FEVER
• BLEEDING/ EASY BRUISING
• Bone pain; lymphadenopathy; cough; headache or
diaphoresis
• MYELOID SARCOMA
– RARE extramedullary tumor mass consisting of myeloid blasts
– Skin; lymph nodes, GIT, soft tissues, testis
(AML)
• PHYSICAL FINDINGS
– FEVER, HEPATO-SPLENOMEGALY, LADs, sternal
tenderness, and evidence of infection and
hemorrhage
• OFTEN found AT DIAGNOSIS
– SIGNIFICANT BLEEDING (GI bleeding, pulmonary
or intracranial hemorrhage)
• OCCURS most often in APL
(AML)
• HEMATOLOGIC FINDINGS
– ANEMIA
• Can be severe
• Normocytic normochromic
– Leukocytic abnormalities
• Median WBC count: 15,000/uL
• <5,000/uL in 25%- 40% of patients
• >1000,000/uL in 20% of patients
• Presence of leukemic cells
– <5% of patients have no detectatble leukemic cells in the PBS
(AML)
• HEMATOLOGIC FINDINGS:
– AUER RODS
• Abnormal- rod shaped granules
• Marker for myeloid lineage
– PLATELET ABNORMALITIES
• <100,000/ uL in ~75% of patients at diagnosis
• <25,000/ uL in ~25% of patients
• Morphologic and Functional abnormalities
(AML)
• PRE- TREATMENT EVALUATION
– FUNCTIONAL INTEGRITY OF MAJOR ORGANS
• LIVER AND KIDNEYS
• CARDIAC AND PULMONARY
– PROGNOSTIC FACTORS
– ANTICIPATORY HEMATOLOGIC SUPPORTIVE
MANAGEMENT
• BLOOD COMPONENT REPLACEMENT TREATMENT
– ANTICIPATORY MANAGEMENT OF TREATMENT
COMPLICATIONS
• HYPERURICEMIA; TUMOR LYSIS SYNDROME
(AML)
TREATMENT
• TWO PHASES
1. INDUCTION TREATMENT
• INITIAL GOAL
• INDUCTION OF COMPLETE RESPONSE
2. POST- REMISSION TREATMENT
• FURTHER THERAPY TO PROLONG SURVIVAL AND
ACHIEVE CURE
(AML)
TREATMENT
• INDUCTION CHEMOTHERAPY
– CYTARABINE
• S- phase cell cycle specific ANTI- METABOLITE
– Interferes with DNA synthesis
– ANTHRACYCLINE
• Daunorubicin, Idarubicin, Mitoxantrone
• DNA intercalators
– TOPOISOMERASE II INHIBITOR  DNA breaks
(AML)
TREATMENT
• ADVERSE EFFECTS of INDUCTION TREATMENT
– HEMATOLOGIC TOXICITY
• ANEMIA, NEUTROPENIA, THROMBOCYTOPENIA
• HIGH- DOSE CYTARABINE TREATMENT
– PULMONARY TOXICITY
– CEREBELLAR TOXICITY
• HIGH DOSE CYTARABINE
• RENALLY- IMPAIRED PATIENTS
• OLDER PATIENTS
(AML)
TREATMENT
• POST- REMISSION THERAPY
– ERADICATE RESIDUAL LEUKEMIC CELLS
– PREVENT RELAPSE AND PROLONG SURVIVAL
– BASED ON AGE AND CYTOGENETIC and
MOLECULAR RISK
(AML)
TREATMENT
• POST- REMISSION THERAPY
– YOUNGER PATIENTS:
• INTENSIVE (HDCT) CHEMOTHERAPY AND ALLOGENIC OR
AUTOLOGOUS HEMATOPOIETIC STEM CELL
TRANSPLANTATION (HSCT)
– OLDER PATIENTS:
• HIGHER INCIDENCE OF TOXICITY
• ATTENUATED CHEMOTHERAPY REGIMENS: LESS EFFECTIVE
• ALLOGENIC HSCT is strongly recommended
(AML)
TREATMENT
• SUPPORTIVE CARE
– PLATELET TRANSFUSIONS
• TO MAINTAIN PLATELET COUNT >10,000/uL
– HIGHER LEVEL IN FEBRILE PATIENTS AND THOSE with DIC
– RBC TRANSFUSION
• KEEP Hgb >80g/L IN THE ABSENCE OF BLEEDING, DIC,
or CHF
*LEUKODEPLETED BLOOD PRODUCTS SHOULD BE USED
TO AVERT ALLOIMMUNIZATION AND FEBRILE REACTIONS
(AML)
• SUPPORTIVE CARE:
– NEUTROPENIA
• ANC <500 u/L or <1000/uL AND PREDICTED TO DECLINE
TO <500 u/L OVER THE NEXT 48 HRS
• HIGH RISK FOR INFECTION
– PROPHYLACTIC ANTIBIOTICS (QUINOLONES) MAYBE BENEFICIAL
• FEBRILE NEUTROPENIA
– MANAGED AS SEPSIS
– INITIAL Tx: BROAD SPECTRUM Antibiotic with coverage for GNB
including Pseudomonas aeruginosa
» CARBAPENEMS, ANTI- PSEUDOMONAL PENICILLINS
(PIPERACILLIN- TAZOBAXTAM) AND CEPHALOSPORINS
(CEFEPIME OR CEFTAZIDIME) +/- AMINOGLYCOSIDES
(AML)
• SUPPORTIVE CARE
– GROWTH FACTORS (G-CSF and GM-CSF)
• GIVEN TO PATIENTS POST CHEMOTHRAPY TO PREVENT
NEUTROPENIA
– SHORTENED TIME TO NEUTROPHIL RECOVERY
– NOT PROVEN TO DECREASE RATE OF INFECTION NOR
SHORTENED HOSPITALIZATIONS
– NO SURVIVAL BENEFIT
• MAYBE USED IN OLDER PATIENTS, THOSE PATIENTS
WITH INTENSIVE CHEMOTHERAPY, in UNCONTROLLED
INFECTIONS, or in CLINICAL TRIALS
(AML)
• REFRACTORY or RELAPSED AML
– DRUG- RESISTANT LEUKEMIA or BECAUSE OF FATAL
COMPLICATIONS OF BONE AMRROW APLASIA OR
IMPAIRED RECOVERY OF NORMAL STEM CELLS
– TREATMENT: SALVAGE CHEMOTHERAPY +
ALLOGENIC HSCT
– LENGTH of COMPLETE RESPONSE: PREDICTIVE
FACTOR for RESPONSE TO SALVAGE
CHEMOTHERAPY
ACUTE PROMYELOCYTIC LEUKEMIA
– HIGHLY CURABLE SUBTYPE OF AML

• 85% of PATIENTS ACHIEVE LONG TERM SURVIVAL
– VERY RESPONSIVE TO CYTARABINE AND
DAUNORUBICIN
• SERIOUS ADVERSE EFFECT: DIC
– DIC can be averted or prevented with the use of TRETINOIN by
inducing differentiation of leukemic cells with t(15;17)
• TRETINOIN
– APL differentiation syndrome
– Fever, fluid retention, dyspnea, chest pain, pleural and
pericardial effusions, and hypoxemia within first 3 weeks of
treatment
CHRONIC MYELOID LEUKEMIA
CML is a clonal hematopoietic stem cell disease
driven by the BCR- ABL1 chimeric gene product
resulting from the balanced translocation
between the long arms of
chromosomes 9 and 22
T(9;22) (q34;q11.2)
PHILADELPHIA CHROMOSOME (Ph)
• 15% OF ALL CASES OF LEUKEMIA
• MALE: FEMALE RATIO is 1.6:1
• MEDIAN AGE AT DIAGNOSIS: 55- 65 YRS
– Only 3% are <20 yrs
• INCIDENCE INCREASE WITH AGE
• ETIOLOGY:
– NO FAMILIAL ASSOCIATIONS
– NO INCRIMINATED ETIOLOGY EXCEPT FOR EXPOSURE
TO HIGH DOSES OF RADIATION
• PATHOPHYSIOLOGY:
DNA sequences from cellular oncogene ABL1
translocated to the major breakpoint cluster region
(BCR) gene    fusion gene encodes the
oncoprotein BCR-ABL1
BCR-ABL1 oncoprotein has constitutive kinase

activity   excessive proliferation and reduced
apotosis of CML cells
Ph Chromosome
• PHILADELPHIA CHROMOSOME
– DEFINES THE DIAGNOSIS OF CML
– DETECTABLE BY CYTOGENETIC ANALYSIS
• IF NOT DETECTABLE: FLUORESCENCE IN SITU
HYBRIDIZATION (FISH) and molecular studies (PCR) can
detect BCR-ABL1
• SYMPTOMS
– most patients (90%) are in the indolent phase
• Non- specific and manifestations of anemia and
splenomegaly
– Fatigue
– Malaise
– Weight loss
– Early satiety and/ or abdominal left upper quadrant pain
• Veno- occlusive events from severe thrombocytosis or
thrombocytosis
– Priapism, MI, venous thrombosis or CVD
• PHYSICAL FINDINGS
– SPLENOMEGALY: most common finding (20-70%)
– HEPATOMEGALY; LYMPHADENOPATHY; and
EXTRAMEDULLARY DISEASE
– SIGNS of HIGH TUMOR BURDEN
• BLEEDING
• THROMBO-EMBOLIC EVENTS
– HIGH BASOPHIL COUNTS
• HISTAMINE OVERPRODUCTION: FLUSHING; DIARRHEA;
EXCORIATIONS FROM PYRURITUS
HEMATOLOGIC and BONE MARROW FINDINGS
• LEUKOCYTOSIS
– 10-500 X 109/L with SHIFT TO LEFT differential count
• PREDOMINANCE OF NEUTROPHILS
• PRESENCE OF BLASTS (usually <5%)
• THROMBOCYTOSIS
– COMMON
– THROMBOCYTOPENIA IS RARE AND SUGGESTS A
WORSE PROGNOSIS
HEMATOLOGIC and BONE MARROW FINDINGS
• ANEMIA
– PRESENT in 1/3 of PATIENTS
• BONE MARROW
– HYPERCELLULAR
– MARKED MYELOID HYPERPLASIA
– HIGH MYELOID-TO-ERYTHROID RATION (15-20:1)
– BLASTS <5%
– INCREASED RETICULIN FIBROSIS
• DIAGNOSIS of CML:
– DOCUMENTATION of the presence of
PHILADELPHIA CHROMOSOME
• PRESENT IN 90% OF PATIENTS
– CYTOGENETIC ANALYSIS of BONE MARROW
ASPIRATE
– FISH and PCR diagnostic techniques
• Aid in the diagnosis of CML
• ACCELERATED PHASE or BLASTIC PHASE CML
– Progression of CML associated with leukocytosis
and therapy resistance with more severe clinical
manifestations
– Poor prognosis
– >30% PERIPHERAL or MARROW BLASTS OR
PRESENCE OF SHEETS of BLASTS in
EXTRAMEDULLARY DISEASE
PROGNOSIS
A. CHRONIC PHASE CML B. ACCELERATED PHASE CML

• TREATMENT
– TYROSINE KINASE INHIBITOR THERAPY in the form of
IMATINIB MESYLATE (2001) has revolutionized the
treatment and prognosis of CML
– GOAL of TREATMENT
• FUNCTIONAL CURE: SURVIVAL with CML similar with
survival among normal individuals
– convert a symptomatic disease to an indolent or asymptomatic
chronic disease
– THERAPEUTIC ENDPOINT:
• ACHIEVEMENT OF COMPLETE CYTOGENIC RESPONSE by 12
months
• ALLOGENIC STEM CELL TRANSPLANT
– CURATIVE TREATMENT MODALITY
– LONG TERM SURVIVAL RATE of 40- 60% when
implemented in the chronic phase
– 5-10 years OS: 50- 60%
– ASSOCIATED WITH SERIOUS ADVERSE EVENTS
– USED Aas SECOND or THIRD LINE treatment in the
POST- TKI ERA
• OTHER THERAPEUTIC AGENTS USED
– INTERFERON α
• USED TO BE THE STANDARD TREATMENT PRE- TKI ERA
– HYDROXYUREA
• SAFE AND EFFECTIVE AGENT to REDUCE INITIAL CML
BURDEN (temporary measure)
– BUSULFAN
• CHEMOTHERAPY PRIOR TO ALLOGENIC HSCT
– SPLENECTOMY
• Occassionally considered to alleviate symptoms of
hypersplenism and/ or splenomegaly
MYELODYSPLATIC SYNDROMES (MDS)
• HETEROGENOUS GROUP OF HEMATOLOGIC
DISORDERS CHARACTERIZED BY:
1. CYTOPENIAS DUE TO MARROW FAILURE
• “LOW RISK” MDS
2. HIGH RISK FOR DEVELOPMENT of ACUTE MYELOID
LEUKEMIA (AML)
• “HIGH RISK” MDS
• Disease of the ELDERLY
– MEAN AGE of ONSET >70 YEARS
• MALE PREDOMINANCE
• INCIDENCE: 35 to > 100/ million
• CLINICAL FEATURES:
– ANEMIA dominates the early course
– SYMPTOMS
• Weakness, fatigue, and dyspnea
• ~50% are asymptomatic at diagnosis
– PHYSICAL FINDINGS
• ANEMIA: pallor, tachycardia
• SPLENOMEGALY in 20% of patients
• SWEET SYNDROME: febrile neutrophilic dermatosis
• PERIPHERAL BLOOD FINDINGS:

– ANEMIA
• In bicytopenia or pancytopenia
– MACROCYTOSIS is common
– Platelets are large and lack granules
– Neutrophils are hypogranualted
– WBC count is usually normal or low
• BONE MARROW
– NO SINGLE CAHARACTERISTIC MORPHOLOGIC FEATURE to
DISTINGUISH MDS
– NORMAL or HYPERCELLULAR
• in 20% of patients, it is hypocellular and can be confused with
aplasia
– COMMONLY OBSERVED FINDINGS
• DYSERYTHROPIETIC CHANGES and RINGED SIDEROBLASTS in
erythroid lineage
• HYPOGRANULATION and HYPOSEGMENTATION in
GRANULOCYTIC PRECURSORS
• INCREASED MYELOBLASTS and MEGAKARYOCYTES
• PROGNOSIS
– VARIABLE
• BETTER for patients with 5q- or SIDEROBLASTIC
ANEMIA
• WORSE for patients with REFRACTORY ANEMIA with
excess BLASTS or severe PANCYTOPENIA associated
with MONOSOMY 7
– MOST PATEINTS DIE AS A RESULT OF
COMPLICATIONS OF PANCYTOPENIA (MARROW
FAILURE) and NOT DUE TO LEUKEMIC
TRANSFORMATION
• TREATMENT:
– HEMATOPOIETIC STEM CELL TRANSPLANTATION
(HSCT) is the ONLY TREATMENT that can offer CURE
– EPIGENETIC MODULATORS
• AZACITIDINE and ECITABINE
• Act through demethylating mechanisms to alter gene
regulation and allow differentiation of abnormal MDS stem
cells to mature blood cells
– LENALIDOMIDE
• EFFECTIVE in REVERSING ANEMIA
• LESS TOXIC
PRIMARY MYELOFIBROSIS (PMF)
• CHRONIC PMF or idiopathic myelofibrosis,
agnogenic myeloid metaplasia, or myelofibrosis
with metaplasia
• CHARACTERIZED by MARROW FIBROSIS,
EXTRAMEDULLARY HEMATOPOIESIS and
SPLENOMEGALY
• Primarily affects MEN in their 6th decade or later
• LEAST COMMON of chronic MPN and DIFFICULT
TO DIAGNOSE
DISORDERS CAUSING MYELOFIBROSIS
• ETIOLOGY is UNKNOWN
– NON- RANDOM CHROMOSOME ABNORMALITIES ARE
PRESENT BUT THERE ARE NO SPECIFIC CYTOGENITIC
ABNORMALITIES
– JAK2 V67F IS PRESENT IN ~50% OF PATIENTS
• FIBROSIS
– ASSOCITED WITH THE OVERPRODUCTION OF TRANSFORMING
GROWTH FACTOR β AND TISSUE INHIBITORS OF
METALLOPROTEINASES
• OSTEOSCLEROSIS
– OVERPRODUCTION OF OSTEOPROTOGERIN, AND OSTEOCLAST
INHIBITOR
– NON- SPECIFIC SYMPTOMS
• NIGHT SWEATS, FATIGUE AND WEIGHT LOSS
– MANY ARE ASYMPTOMATIC AT PRESENTATION
– INCIDENTAL FINDING ON ROUTINE BLOOD TESTS
– SPLENIC ENLARGEMENT
– ANEMIA is usually MILD
– INASPIRABLE MARROW
– EXUBERANT EXTRAMEDULLARY HEMATOPOIESIS
• PORTAL, PULMONARY AND INTRACRANIAL HYPERTENSION
• DIAGNOSIS
– Diagnosis of PMF is ONE of EXCLUSION
– CLINICAL FEATURES CAN BE OBSERVED IN PV or CML
and MANY DISORDERS HAVE FEATURES THAT
OVERLAP WITH PMF
– BONE MARROW
• Hypercellular with trilineage hyperplasia and, in particular,
increased numbers of megakaryocytes in clusters and with
large dysplastic nuclei
– Presence of autoimmune abnormalities
• (+) ANA, Rf, (+) Coomb’s test
• COMPLICATIONS
– Increasing MARROW FAILURE with TRANSFUSION-
DEPENDENT ANEMIA and increasing ORGANOMEGALY due
to extramedullary hematopoiesis
• TREATMENT
– NO specific THERAPY
– CORTICOSTIROIDS can ameliorate anemia and constiutional
symptoms
– Low dose THALIDOMIDE can be given for thrombocytopenia
– ALLOPURINOL for hyperuricemia
– RT for bone pain
ESSENTIAL THROMBOCYTOSIS
• ESSENTIAL THROMBOCYTOPENIA; idiopathic
thrombocytosis; primary thrombocytosis; or
hemorrhagic thrombocytopenia
• CLONAL DISORDER of UNKNOWN ETIOLOGY
involving multipotent progenitor cell leading to
the overproduction of platelets
• FEMALE predominance and can occur at ANY AGE
• NO CLONAL MARKER
– DIAGNOSIS is DIFFICULT
– INCIDENTAL FINDING ON ROUTINE EXAMINATION
– NO SPECIFIC SIGNS or SYMPTOMS
• HEMORRHAGIC or THROMBOTIC TENDENCIES: bleeding
• MICROVASCULAR OCCLUSIVE EVETS
– OCULAR MIGRAINE, TIA
• MILD NEUTROPHILIC LEUKOCYTOSIS
• HYPERKALEMIA
– NO CHARACTERISTIC PLATELET FUNCTION
ABNORMALITY
• BONE MARROW
– MEGAKARYOCYTE HYPERTROPHY and HYPERPLASIA,
as well as MARROW CELLULARITY
• If reticulin is increased, another diagnosis should be
considered
• DIAGNOSIS
– Difficult as thrombocytosis can be a manifestation of
another disease
– ~50% have JAK2 V617F
• CYTOGENETIC TESTS ARE DONE TO EXCLUDE CML and PV
• TREATMENT
– SURVIVAL of patients is NOT DIFFERENT to the general
population
– NO TREATMENT for ASYMPTOMATIC patients
– Salicylates (ASA)
• May be given especially if symptomatic
• For PLATELET COUNTS > 1 x 106/uL, ASA can cause acquired von
Willebrand’s disease
– May be treated with aminocaproic acid
– PLATELETPHARESIS
– ANAGRELIDE and HYDROXYUREA are other drugs given for
occlusive complications
THANK YOU!!!
• READ HPIM for more details….

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MYELOID NEOPLASMS

DR. MARY GAY BULIYAT,

– HIGHLY CURABLE SUBTYPE OF AML

BCR-ABL1 oncoprotein has constitutive kinase

A. CHRONIC PHASE CML B. ACCELERATED PHASE CML

• PERIPHERAL BLOOD FINDINGS:

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