Hematologic Diseases General Introduction of Hematology

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Hematologic Diseases

• General Introduction of Hematology

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Components and functions of blood

• Two major components
• blood cells(cellular fraction)
• plasma (non-cellular fraction)
• Primary functions
• red blood cell to transport O2 and CO2
• neutrophils to destruct microorganisms
• lymphocyte to mediate immune reaction

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T cell (thymus dependent ) cell- mediated
immune B cell (BM dependent ) humoral-mediated
immune Monocytes to phagocytize and modulate
immune reaction Eosinophils to be involved in
IgE immune reaction Basophils to be involved in
type I hypersensitivity
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Plasma relevant to coagulation factors,
immunoglobulins and complements
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Compositions of hematopoietic organs

• Hematopoietic organs blood, bone marrow,
  liver,spleen, lymph nodes
• Structure of bone marrow
• outside cortex
• inside medulla 50 hematopoietic and 50
  fat tissues
• hematopoietic cells are arranged in cords
  around sinusoid
• mature cell are released into sinusoid
  through endothelia
• cell of sinus
• abundant blood vessles are linked to sinusoid

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• Hematopoiesis in adult
• To be confined to bone marrow and lymphocyte
• Hematopoiesis in fetal
• Yolk sac, liver and spleen
• Extramedullary hematopoisis

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Process of hematopoiesis

• Hematopoietic cell
• Pluripotential stem cell
• CD34/ CD38-
• A small compartment of stem cells
• A large compartment of proliferating cells of
  committed lineage
• A large compartment of maturing cells of both
  myeloid and lymphoid

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• Hematopoietic growth factor
• Erythropoietin(EPO)
• G-CSF,GM-CSF
• Thrombopoietin (TPO), IL-11

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Classification and characteristics of
hemayologic diseases

• Diseases of red blood cells
• Aplastic anemia, IDA, hemolytic anemia,
  megaloblastic anemia, thalassemia, sideroblastic
  anemia
• Diseases of white blood cells
• Leukepeania, leukecytosis, leukemia,
  lymphoma, myeloma, myelodisplastic syndrome(MDS)
• Diseases of bleeding and thrombosis ITP, DIC,
  hemophila

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Characteristics of blood diseases

• Numerous types
• Low incidence
• High mortality
• Poor prognosis
• Multi-system involved
• Lab-test dependent diagnosis

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Diagnosis of hematopoietic diseases

• History symptoms, period of diseas, inductive
  factors, personal history, occupation history,
  family history, diet habit, drug take-in history,
  age, sex
• Physical examination pallor, purpura, fever,
  sternum tenderness, lympho node, hepatomegaly,
  splenomegaly
• Laboratory tests BRT, morphology, bone marrow
  biopsy, DIC examination, hemolytic examination,
  biochemical and immunologic examination,
  chromosome banding technique, FCM detection

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The progresses in hematologic diseases

• Immunotherapy leukemic vaccine, dendritic
  vaccine, cytokine gene-transduced vaccine,
  recombinant hematopoietic growth factor
  rIL-2,rIL-11,rTPO, rEPO,rGM-CSF,
• Hematopoietic stem cell transplantation
  BMT,peripheral stem cell transplantation, cord
  blood transplantation
• Gene therapy hemophilia, thalassemia, leukemia,
  gene chip

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Introduction of anemia

• Concept of anemia
• Anemia is a syndrome, not a name of a disease
• Anemia is a common symptoms in many diseases
• In anemia status, Hb and RBC are lower than
  normal
• Decreases of Hb is more important in judge
  degree of anemia

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• Causes and mechanisms of anemia
• The injury of progenitor of erythrocyte
  physical,chemical, viruses factors
• The lack of hematopoietic factors
  ferritin,vitB12, folic acid,vitB6,vitC
• The lack of bone marrow tissues leukemia,
  lymphoma,MM, solid tumor infiltrate to bone
  marrow
• Increase of red blood cell destruction
  hereditary and acquied hemolytic anemia abnormal
  of RBCmembrane, Hb, RBC enzymes

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Classification of anemia

• According to the causes and mechanisms of anemia
  to type
• Decrease of red blood cell production(AA,
  IDA,leukemia)
• Increase of red blood cell destruction
• (hemolytic anemia)
• Acute or chronic blood loss, GI tumor
  hypermenorrhea, hemorrhiod, hook-worm

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• Accoding to morphology
• Microcytic hypochromic anemiaIDA
• Megalocytic anemia deficiency folic acid and
  vitB12
• Normal erythrocytic anemia AA, hemolytic anemia

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Clinic features of anemia

• Shortness of breath on exertion
• Palpitation
• Tiredness, weakness or fatigue
• Faintness tinnitus
• Headache
• Spots before the eyes

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• Factors influencing symptoms of anemia
• The rapidity of onset and severity of anemia
• The ability of patients to make cardiovascular
• compensations
• Age old person with severe symptoms
• Sex female with severe symptoms

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Diagnosis of anemia

• Whether anemia exist
• How severe anemia is
• Original causes diseases of anemia

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Treatment of anemia

• Primary diseases and causes
• Supportive treatment blood transfusion
• Hematopoietic factors EPO, iron, folic acid
  vitb12
• Immune suppressor CsA, Prednision,
• Splenectomy HS AIHA
• BMT AA, PNH, MDS

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Iron Deficiency Anemia(IDA)
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Concept and characters of IDA

• IDA is usually caused by chronic blood loss or
• lack of hematopoietic nutrient ferritin by
• malabsorption.
• The morphologic characteristic of IDA is
• microcytic hypochromic anemia.
• IDA is one of the most common nutritious anemia
• in the world.

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Iron metabolisms

• In general, iron metabolism depends on a precise
  balance between iron absorption and excretion.
• Iron is supplied by food,10 to 20 mg of iron is
  presented in the average daily food, which is
  enough to healthy adult male and nonmenstrating
  female.
• Iron absorption occurs mainly in the duodenum and
  proximal jejunum. Gut mucosal cells regulate
  absorption by storage iron.
• Normally, 5-10 dietary iron is absorbed.

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• Iron enters the mucosal cells is either bound to
  transferrin or transported into the portal
  circulation or remains in the mucoscal cell .
  Transferrin delivers either to bone marrow
  tissues for Hb synthesis or to liver to store.
• The reticuloendothelial system phagocytoses
  senescent red blood cells. About 2/3 iron
  obtained by metabolism of Hb within the
  reticuloendothelial system binds rapidly to
  plasma transferrin for transport to marrow
  reuseness. The others is stored within the
  macrophage as ferritin or hemosiderin.

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• Approximately 1mg of iron is lost daily by the
  adult male and nonmenstruating female via sweet,
  urine, stool. Menstruating females lose an
  additional 10-30mg of iron with each menstrual
  period.
• Daily needed iron
• adult male 1mg/d
• children 2mg/d
• menstruating female 3mg/d
• pregnant and breast feeding woman 4mg/d

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Causes and primary disease of IDA

• Inadequate dietary iron related to inappropriate
  food , Iron deficiency occurs more commonly in
  populations whose diets often lacking of meats.
• Increased iron requirement woman with
  menstruation and pregnancy. Infancy and
  adolescence when growth is rapid.
• Decreased iron absorption partial and total
  gastrectomy and achlorhydria.

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• Blood loss predominant cause of iron deficiency
• young woman -menstrual blood loss during a
  normal menstrual period, approximately 32mg iron
  may be lost(21)
• old man and non-menstrual woman-gastro-intensti
  nal blood loss GI tumor,
• young man blood loss peptic ulcer,
  hemorrhoid,
• children blood loss hookworm disease,

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Clinical features of IDA

• General symptoms of anemia palpitation,
  shortness of breath, tiredness, headache
• Specific symptoms of IDA spoon-shaped
  nails(koilonychia) with dry , brittle, ridged
  changes
• A hunger for unusual substances ice, clay,
  hair,
• Digestive symptoms sore tongue, atrophy of the
  gastric mucosa, plummer-vinson syndrome

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Laboratory tests of IDA
BRT Hb decrease, WBC and platelet are
normal Bone marrow microcytic hypochromatic
changes in morphology, intracellular iron is
lt15, extracellular iron is negative Iron
metabolism index serum iron decrease, serum
ferritin protein decrease, total iron binding
capacity increase
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Diagnosis and differential diagnosis

• History
• Morphologic characteristics of RBC
• BM examination and iron staning
• Serum iron and serum ferritin level
• Diagnostic treatment with iron

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Treatment of IDA

• Treatment of primary diseases
• Treatment with iron ferrintherapy
• Oral iron ferrous sulfate 300mg tablet daily
  and gradually increase to three time daily post
  meal
• Injection iron iron dextran im, 50-100mg
  daily,total iron needed
• ( Hb15g/dl -patients Hbg/dl ) x300mg500mg

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Hemolytic anemia
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Introduction

• Hemolytic anemia results from an increased rate
  and speed in RBC destruction.
• BM has the capacity to compensate production of
  RBC from 6-8 folds. Compensated hemolytic disease
  has a persistently elevated reticulocyte count
  with a normal Hb.
• In situ hemolysis RBC are destructed in BM
  before they are released into blood (lt10)

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• Extravascular hemolysis RBC are phagocytosed by
  reticuloendothelial system and removed from the
  circulation because of inflexible prematurely.
  AIHA,
• HS,
• Intravascular hemolysis RBC are actually lysed
  in the circulation and blood vessels.
• PNH, DIC

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physiopathology of hemolytic anemia

• Increased hemolysis
• Increased serum bilirubin
• Increased urine urobilinogen
• Decreased serum haptoglobin
• Increased production of RBC
• Reticulocytosis and polychromasia
• Erythroid hyperplasia in BM

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• RBC damage
• Morphology fragment, micro-spherocytes,
  spherocytes
• Increased osmotic fragility
• Short RBC survival time
• Hemoglobinemia and hemoglobinuria

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Classification of hemolytic anemia

• RBC intrinsic abnormal
• Memberane (hereditary spherocytosis)
• Enzyme (G-6PD deficiency)
• Hemoglobinopathy( sick cell diseases)
• Globin synthesis (thalassemia)

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• RBC extrinsic abnormal(usually acquired)
• Abtibody induced (coombs positive hemolytic
  anemia)
• worm antibody and cold antibody
• Drug induced (penicillin)
• Toxin mediated (burns, sepsis)
• Mechanical hemolysis(heart valve surgery)

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Clinic features of hemolysis

• General symptoms of anemia
• Jaundice
• Hemoglobinuria
• Acute hemolysis high fever, cold, pain of back,
  nausea and vomit, occurs rapidly with
  intravascular hemolysis
• Chronic hemolysis splenomegaly, uncler on ankle
  skin and bile pigment stone, occurs slowly with
  extravascular hemolysis.

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Laboratory tests

• BRT Hb decrease with specific morphology changes
  in mature RBC, reticulocytosis( at least gt5)
• BM erythroid hyperplasia
• Short RBC survival time
• Specific test for hemolytic anemia coombs test,
  Ham test, Rous test,
• High free Hb concentration in serum
• Increased serum indirect bilirubin and
  urobilirubin

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Diagnosis of hemolytic anemia

• Three steps
• Be sure whether anemia Is hemolytic anemia?
• Exact kind of hemolysis
• What is primary diseases led to hemolytic anemia?

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Treatment of hemolytic anemia

• Corticosteroid prednisone, DXM,
  methylprednisolone to be used in autoimmune
  hemolytic anemia(AIHA)
• Dosage 30-60mg/d to Hb elevated to normal,
  gradually drop down once a week and then maintain
  with low dose (5-10mg/d) for 3-6 months
• Side-effect higher blood suger,
  gestro-uncler,hypertention, sodium-water retention

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• Immunsuppressor CsA, danazol, azathioprin
• Splenectomypatients have no therapuetic effect
  to corticosteroid after 3-6 months, or patients
  with HS
• Blood transfusion washed RBC, RBC , transfusion
  should be matched well in ABO and Rh type.
• Hematopietic stem cell transplantation

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Myelodysplastic syndromes
(MDS)
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Concept of MDS

• MDS are characterized by slow development of a
  refractory anemia to standard anti-anemia
  therapy.
• MDS is divided into primary (de novo)and
  secondary MDS.
• Patients with secondary MDS usually have prior
  treatment with radiation therapy or chemotherapy
  or both for primary neoplasma, such as lymphoma,
  solid tumor.

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• The incidence of MDS is higher in 50-60 year old
  person.
• The patients present with the insidious onset of
  anemia.
• Most of cases of MDS finally transfer to
  leukemia. Therefore, MDS is also called as
  preleukemia stage.

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Causes and mechanisms

• Following factors injury stem cell and lead to
  develop of MDS
• Drugs and chemicals
• Radiation
• infection

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Clinical features of MDS

• Fatigue and tiredness
• Infection, fever, bleeding
• Leukocytopeania, thrombocytopeania,
  anemiapancytopeania
• Plapitation, short of breath

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Classification of characteristics

• FAB classification of subtypes
• Type blood bone
  marrow
• RA(56) blast lt 1 blast lt 5
• RAS(21) blast lt1 blast lt5
• 
  sideroblast gt15
• RAEB(10) blast lt 5 blast 5-20
• RAEB-T(1) blast gt5 blast 20-30

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Diagnosis of MDS

• Pancytopeania
• Refractory anemia, infection, bleeding
• malhematopoiesis at least two cell lines
  myeloid or erythroid or megakaryocyte line in
  bone marrow
• Bone marrow biopsy demonstrated ALPC (abnormal
  location of premarture cell) and increased lattic
  fibers.
• Hematopoietic sites with island of
  pre-erythrocytes or increased erythroblast.
• Exclude the diagnosis of other anemia, leukemia
  and myelofibrosis.

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?1 ?????
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?2 MDS-RA(??????)
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?3 MDS-RA(??????)
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?4 MDS-RA(????????)
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? 5 MDS-RAS
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?6 MDS-RAEB
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?7 MDS-RAEB-T
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?8 CMML
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Treatment of MDS

• Supportive treatment blood transfusion,
  antobiotics, GM-CSF
• Inductive differentiation treatment all
  trans-retinoic acid (RA) 40-60mg/d , 2-3 months
• Low dosage chemotherapy cytarabine (Ara-C)
  10-25mg/d i.v 2-3 weeks
• Stamulate erythropoiesis EPO 2000-3000U/d. I.H
  2-3 months, stanozolol 6mg/d, po 2-3 months
• Allo-BMT or allo- PBSCT

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Leukemia
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What is leukemia

• Stem cell disease malignant cloning
• leukemic stem/progenitor cells production
• differentiation/maturation stop infiltration
  of
• tissues/oranges
• lymphoid (B/T) myeloid (MM/E/Meg)
• immature mature immature
  mature
• acute chronic acute
  chronic

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Classification of leukemia

• According to maturation and natural disease
  stage
• Acute leukemia (AL)
• low grade differentiation with the most of
  blast /
• immature cells, disease develops very fast
  and prognosis is very bad, mean natural disease
  stage is about 3 months or so.
• Chronic leukemia (CL)
• high grade differentiation with the most near
  mature /mature cells, disease develops relatively
  slowly and
• prognosis is comparatively good, mean
  natural disease stage is several years .

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• According to involved cell line
• Acute leukemia
• Acute lymphoblast leukemia(ALL)L1-L3
• Acute myelocytic leukemia (AML) M1-M7
• Or acute non-lymphocytic leukemia (ANLL)
• Chronic leukemia
• Chronic myelocytic leukemia (CML)
• Chronic lymphocytic leukemia (CLL)
• Hairy leukemia (HL)

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Current status of leukemia in China

• Incidence of leukemia is 2-3/100,000 with the
  tendency of increasing yearly!
• Low than Europe and America, same as Asian
  countries
• Acute gt chronic(5.51)
• ANLL gt ALL(adult / children)
• CML gt CLL

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Causes and mechanisms

• Virus etiology RNA virus (RT-virus),
• HTLV-1,
• Radiationx-ray, r-ray, isotope,
• Chemicals and drugs anti-tumor drugs,
  antibiotics chloramphenicol, chemicals benzene,
• Hereditory factorsfamily leukemia, chromosome
  changes, tumor gene expression
• Develop from other blood diseasesMDS, PNH, ML,
• MM,MF,

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Acute leukemia

• Classification of acute leukemia (FAB)
• ALL subtypes L1-L3
• L1 small lymphoblast and immature lymphocytes
  with comparative good prognosis
• L2 small and large lymphoblast and immature
  lymphocytes with comparative bad prognosis
• L3 large lymphoblast and immature lymphocytes
  with very bad prognosis

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ANLL subtype M1-M7 M1 acute myelocytic
leukemia without differentiation,
myeloblast gt90 M2 acute myelocytic leukemia
with partial differentiation, myeloblast
between 31- 89 M3 acute premyelocytic
leukemia(APL) M4 acute myelomonocytic
leukemia(AMML) M5 acute monocytic leukemia M6
acute erythroleukemia M7 acute megakaryocytic
leukemia
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• MIC typing MICMB typing
• Morphology FAB typing
• Immunology monoclonal antibodies typing
• Cytogenetics chromosome analysis and
  typing
• Molecular Biology gene detect and analysis
• APL M3b CD33/ CD13t(15,17) PML-RARa
• M3b
• CD33/ CD13
• t(15,17)
• PML-RARa

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Clinical manifestations of acute leukemia

• Four clinical features
• Anemia aggressive
• Bleeding multi-site
• Fever infectious
• Infiltration extensive and multi-system
• liver, spleen, lympho node, gum, eye, glands,
• breast, bone, CNS, testicle, lungs,
  heart,chloroma

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• ???????????
• ?? WBC ??, ???????
• ??, ???????
• ??, ???????
• HB, RBC ??????
• Plt ????,

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Laboratory examination of acute
leukemia

• BRT
• WBC increase with many leukemic cellsin PB
• WBC normal with little leukemic cells in PB
• WBC decrease without leukemic cells in PB
• Hb and RBC decrease in different degrees
• platelet sharply decrease, lt60x109/L

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• Hyperleukocyte leukemia
• acute leukemia with WBC gt 100x109/L
• Hyperblast crisis
• acute leukemia with blast cells
• gt100x109/L

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• myelogram
• Extreme cellularity
• Leukemic blast cell gt30
• Depression of erythrocyte
• Depression of megakaryocyte
• Abnormal morphology and structure Auer body,
  nuclear changes

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Characters of cytohistochemistry in AL
ALL
AML
AMoL POX -
- - or PAS
- - or
- or NSE -
- or

NaF cant inhibit NaF can
inhibit ALP -
or - or
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• Immunologic examination
• B cell CD10, CD19, CD20,CD22, TdT,
• HLA-DR
• T cell CD3, CD7, TdT,
• granulocyteCD13, CD33,HLA-DR, MPO
• monocyte CD14
• megakaryocyte CD41, CD42,PPO
• stem cell CD34/CD38-

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• Chromosome and gene detect
• M2 t(821)(q22q22) AML/ETO
• M3 t(1517)(q22q21) PML/RARa
• M4Eo inv/del(16)(q22) CBFB/MYH11
• M5 t/del(11)(q23) MLL/ENL
• L3(B) t(814)(q24q32) MYC and IgH
• ALL(5-20) t(922)(q34q11) bcr/abl

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• Biochemistry test
• serum uric acid increase uricemia
• coagulation factors deficiency
• CSF high pressure of CSF
• WBC increase )gt0.01x109/L with
  leukemic
• cells in CSF
• protein increase gt450mg/L
• glucose decrease

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Diagnosis and differential diagnosis

• MDS pancytopenia with excess blast and immature
  cells , but lt30
• AA pancytopenia without blast and immature cells
  
• ITP thrombocytopenia along, megakaryocyte
  increase obviously, PAIg
• Megaloblast anemia erythroleukemia(M6)
• Acute agranulocytosis premyelocyte increases
  without Auer body

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Treatment of acute leukemia

• non-curable give-up treatment
• curable combined chemotherapy
  
• clinical complete curable
  immunotherapy
• complete eliminated curable HPST

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• Chemotherapy
• The principles of chemotherapy
• earlyas early as possible
• combinationCCSACCNSA
• high dosage dosage effect relationship
• interval 2-3W
• repeatto eliminate minimal residual
• leukemic cells
• individuationexperience and drug
• sensitivity test in
  vitro

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• Steps of chemotherapy
• Inductive remission treatment (IR) from
  diagnosis to get complete remission by 1-2
  protocols. A critical therapeutic step.
• Consolidation and intensification treatment
  (CI) 6-9protocols after IR to eliminate minimal
  residual leukemic cells. An inevitable step.
• Maintenance treatment(MT)2-3years treatment
  after CI. An important step to prevent relapse
  and prolong disease free survival time (DFS).

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• Chemotherapy protocols for ALL
• IR CI
  MT
• VP EA
  chose CI
• V2mg/d1,8,15,22 E100mg/d1-3 protocols,
  
• P60mg/d1-28 A150mg/D1-7 last for
  3-5
• VDPVP VLDP
  years
• D40mg/d1-3 HD-MTX
• VLDPVDP 1.5-3.0g/d1
• L10000U/d19-28

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• Chemotherapy protocols for ANLL
• IR CI
  MT
• DA D40mg/d1-3, DA
  depends,
• A150mg/d1-7 MD-Ara-c
  follow-up,
• HA H4mg/d1-7 MAA
  retreat when
• A150mg/d1-7 M8mg/d1-3
  relapse
• HOAP HA A150mg/d1-7
• V2mg/d1 MEM
• P60mg/d1-7 E100mg/d1-7

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Treatment for special type of AL APL(M3)
All-trans retinoic acid (ATRA) or Arsenic
low dosage HA
anti-DIC Elderly leukemia low dosage longer
treatment stage CNS leukemia subarachnoid cave
injection skull
radiation Testicle leukemia radiation
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• Supportive treatment
• Anti-infection anti-bacterial, anti-virus,
  anti-fungi
• Improve anemia blood transfusion
• Control bleeding platelet transfusion,
  coagulation
• factors
  transfusion, fresh plasma
• Supply nutrition amino-acid, albumin, vitmines ,
  
• trance elements,
  immunoglobulin
• Prevent uricemia and renal lesion

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Hematopoeitic stem cell transplantation
Auto-HST auto-BMT auto-BM
bank auto-PBHST
auto-PBHS bank
auto-CBHST auto- cord blood bank Allo-HST
allo-BMT (sibling) allo-PBHST
1/4 HLA match
allo-CBHST Unrelated-HST unrelated- BMT
unrelated-PBSCT
HPS bank, 1/10000
unrelated-CBSHT Cord bank
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Chronic myelocytic leukemia

• clinic features
• onset is slowly
• no specific manifestations
• giant spleen
• sternal tenderness
• WBC increase obviously, gt 50x109/L

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• Development of disease
• Chronic phase(CP) 1-3years, disease is stable,
• blast cellslt10
• Accelerate phase(AP)several months to years,
• disease develop fast and blast
  cell gt10
• Blast crisis phase(BC)several months only,
• prognosis is very bad, blast
  cellsgt 20

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• Laboratory test
• BRT
• WBC gt20, gt50, gt100x109/L
• DC myelocyte,metamyelocyte, Nst increase
• Eo, Ba increase
• Hb and Plt decrease in terminal phase

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• myelogram
• extreme hypercellularity, ME increase
• myelocyte, metamyelocyte and Nst increase
• Eo, Ba increase
• erythrocyte and megakaryocyte normal
• ALP activity decrease or negative

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• Cytogenetics and moleculor biology
• t(922)(q34q11)
• bcr-abl fusion gene
• P210 protein
• Biochemistryuric acid increase in
• serum and urine

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• Diagnosis and differential diagnosis
• leukomoid reaction(LR)
• primary diseases severe infection, tumor, TB
• Eo,Ba normal
• ALP ,Ph chromosome-
• Myelofibrosis(MF)
• immature erythrocyte and granulocyte appears
  
• in PB with tear drop like RBC
• Ph and ALP

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• Treatment
• chemotherapy
• single drug hydroxyurea, 3g/d
• myleran6mg/d
• combined chemotherapy
• HAH2-4mg/d,A100-150mg/d
• MA M4-6mg/d
• A 50-100mg/d

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• Blast crisissame as acute leukemia
• Interferon a-IFN 3,000,000-9,000,000U/d
• for 1-2years to get Ph chromosome negative
• Glivec a strong inhibitor of tyrosinase to P210
• leukapheresisto eliminate extra leukemic cells
• radiation in spleen area
• aplenectomy
• allo-BMT or allo-PBSCT

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