急性白血病课件
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anemia,infection,bleeding ❖ Direct and indirect pathophsiologic
pathways causing A.A
Classification
Ⅰ Severe
Ⅱ
Moderate
Abroad
Acute Chronic Home
Epidemiology
Case Presentation
▪ 21 year man ▪ Presented with fatigue,pale,tiredness ▪ Hb 11.0 WBC 2.6,neutrophils 1.1, platelets
45, MCV 104. ▪ B12/folate/ferritin were normal. ▪ Main physical examination was
Aplastic Anemia
(再生障碍性贫血)
Wang li Haematology Department The First Affiliated Hospital
Review
What is the function of bone marrow Hematopoiesis
Red blood cells --- transport oxygen and nutrients White blood cells --- fight infection platelets—help blood clot
Laboratory findings
Acute AA
Chronic AA
Blood count（x109/L）
granulocytes
<0.5
>0.5
platelets
<20
>20
reticulocytes
<15
>15
BM morphology
multiplace hypocellularity Similar findings,
▪ Historically test was Ham’s test; showed red cell lysis by complement activation in acidified serum.
▪ Currently test for absent proteins on cell surface. CD55 and CD59
General Introduction
The main characters of Aplastic Anemia
❖ Bone marrow haematopoiesis failure, HSC injured
❖ Peripheral blood pancytopenia ❖ Typical symptoms:
▪ Response rates 60-70%
▪ Relapses are common and continued supportive care needed.
▪ Up to 50% of relapsed patients will respond to 2nd course of immunosuppressive therapy
Routine therapies for anemia fail to work.
Differential Diagnosis -- Pancytopenia
▪ Myelodysplastic syndrome --dysmorphic features
▪ Paroxysmal nocturnal haemoglobinuria (PNH)
Marrow Fat
Aplastic anemia (HP)
BM morphology of Acute AA Recover phase
Diagnosis
Diagnostic standard of AA (1987)
Sign
BM
Diagnosis
Peri Blood
exclusion
Diagnosis
unremarkable.
Case Presentation
▪ Marrow aspirate hypocellular ▪ Marrow biopsy hypocellular
▪ Diagnosis? Treatment?
Thank you
John Radcliffe Hospital
sulfonamide, NSAIDs etc)
chemicals (benzene, insecticides etc) ➢Physical factors
radiation, X ray
➢Biological factors
viruses, severe bacterium, infection
➢Other pathogenic factors ➢Unknown
Pathogenesis
Failure in hematopoietic stem or progenitor cells
Defect in microenvironment of haematopoiesis
Immuno-mediated bone marrow failure
Diagnostic standard of AA (1987)
Decreased peripheral blood count， absolute number of reticulocytes reduced
Without megalosplenia
Diagnosis
BM morphology
Aplastic anemia biopsy
Hematopoietic elements in this bone marrow biopsy are markedly reduced. Of course, RBC, platelets and
granulocytes will often be diminished.
▪ Hypoplastic Acute Leukemia ▪ Others
PNH-- Paroxysmal nocturnal haemoglobinuria
▪ An acquired haematopoietic stem cell defect with predominant haemolytic anaemia.
lack of megakaryocytes lack of E,G increase in marrow fat
focal area active haematopoiesis
increased non-HC
Bone marrow aspiration
Normal BM biopsy
This marrow is taken from a middle aged person, so it is about 50% cellular, with steatocytes admixed with the marrow elements.
donors ▪ Anti-Thymocyte Globulin(ATG) or anti-
lymphocyte globulin (ALG), cyclosporin, methylprednisone ▪ Best results are for combination therapy. ▪ Response is slow, 4-12 weeks to see early improvement
private room face mask hand wash broad-spectrum antibiotics if fever
Immunosuppressive therapy
▪ Indicated for patients > 50 years ▪ Patients with no HLA matched sibling
▪ A descriptive term for the clinical manifestation of haemolysis and haemoglobinuria manifest by dark coloured urine in the morning.
▪ Paroxysmal nocturnal haemoglobinuria.
Moderate or severe hypocellularity found at least one aspiration (biopsy) site. Nonhaematopoietic cells increase in BM.
Diagnosis
Other diseases which may cause decreased blood count are carefully excluded，such as myelodysplastic syndrome(MDS), acute myelogenous leukemia(AML).
Treatment
Supportive Care
Growth Hormones
Immune Suppressive
Therapy
Hematopoietic Stem Cell
Transplantation
Supportive Care
▪ Red cell transfusion ▪ Platelet transfusion ▪ Management of neutropenia
Genetic aspects: HLA associated susceptibility
Seeds Soil
Harmful Insects
Presenting symptoms
Acute AA
Chronic AA
Onset acute, short history Anemia severe, remarkable
Normal BM (high power magnification)
Note the presence of megakaryocytes, erythroid islands, and granulocytic precursors. This marrow is taken from the posterior iliac crest in a middle aged person, so it is about 50% cellular, with steatocytes admixed with the marrow elements.
HSCT-HLA identical sibling HSCT
▪ Age <55 years. ▪ Conditioning with Cyclophosphamide &
antithymocyte globulin, with cyclosporin and methotrexate. ▪ Long term overall survival = 80-90% ▪ Chronic graft versus host disease (GVHD) remains a problem for 25-40% of patients.
slowly onset, longer history
relatively moderate
Bleeding heavy bleeding, multiorgan
Infection more frequent, sepsis
hemorrhage moderate, skin and mucus
moderate, upper respiratory infection
Incidence
China Annual incidence
7.4 per 100,000
1.4 6.0
Acute Chronic
Epidemiology
Distribution
female
male
ቤተ መጻሕፍቲ ባይዱ
Infant
70’s
Etiology
➢Chemical factors: drug (anticarcinogen, chloramphenicol,
pathways causing A.A
Classification
Ⅰ Severe
Ⅱ
Moderate
Abroad
Acute Chronic Home
Epidemiology
Case Presentation
▪ 21 year man ▪ Presented with fatigue,pale,tiredness ▪ Hb 11.0 WBC 2.6,neutrophils 1.1, platelets
45, MCV 104. ▪ B12/folate/ferritin were normal. ▪ Main physical examination was
Aplastic Anemia
(再生障碍性贫血)
Wang li Haematology Department The First Affiliated Hospital
Review
What is the function of bone marrow Hematopoiesis
Red blood cells --- transport oxygen and nutrients White blood cells --- fight infection platelets—help blood clot
Laboratory findings
Acute AA
Chronic AA
Blood count（x109/L）
granulocytes
<0.5
>0.5
platelets
<20
>20
reticulocytes
<15
>15
BM morphology
multiplace hypocellularity Similar findings,
▪ Historically test was Ham’s test; showed red cell lysis by complement activation in acidified serum.
▪ Currently test for absent proteins on cell surface. CD55 and CD59
General Introduction
The main characters of Aplastic Anemia
❖ Bone marrow haematopoiesis failure, HSC injured
❖ Peripheral blood pancytopenia ❖ Typical symptoms:
▪ Response rates 60-70%
▪ Relapses are common and continued supportive care needed.
▪ Up to 50% of relapsed patients will respond to 2nd course of immunosuppressive therapy
Routine therapies for anemia fail to work.
Differential Diagnosis -- Pancytopenia
▪ Myelodysplastic syndrome --dysmorphic features
▪ Paroxysmal nocturnal haemoglobinuria (PNH)
Marrow Fat
Aplastic anemia (HP)
BM morphology of Acute AA Recover phase
Diagnosis
Diagnostic standard of AA (1987)
Sign
BM
Diagnosis
Peri Blood
exclusion
Diagnosis
unremarkable.
Case Presentation
▪ Marrow aspirate hypocellular ▪ Marrow biopsy hypocellular
▪ Diagnosis? Treatment?
Thank you
John Radcliffe Hospital
sulfonamide, NSAIDs etc)
chemicals (benzene, insecticides etc) ➢Physical factors
radiation, X ray
➢Biological factors
viruses, severe bacterium, infection
➢Other pathogenic factors ➢Unknown
Pathogenesis
Failure in hematopoietic stem or progenitor cells
Defect in microenvironment of haematopoiesis
Immuno-mediated bone marrow failure
Diagnostic standard of AA (1987)
Decreased peripheral blood count， absolute number of reticulocytes reduced
Without megalosplenia
Diagnosis
BM morphology
Aplastic anemia biopsy
Hematopoietic elements in this bone marrow biopsy are markedly reduced. Of course, RBC, platelets and
granulocytes will often be diminished.
▪ Hypoplastic Acute Leukemia ▪ Others
PNH-- Paroxysmal nocturnal haemoglobinuria
▪ An acquired haematopoietic stem cell defect with predominant haemolytic anaemia.
lack of megakaryocytes lack of E,G increase in marrow fat
focal area active haematopoiesis
increased non-HC
Bone marrow aspiration
Normal BM biopsy
This marrow is taken from a middle aged person, so it is about 50% cellular, with steatocytes admixed with the marrow elements.
donors ▪ Anti-Thymocyte Globulin(ATG) or anti-
lymphocyte globulin (ALG), cyclosporin, methylprednisone ▪ Best results are for combination therapy. ▪ Response is slow, 4-12 weeks to see early improvement
private room face mask hand wash broad-spectrum antibiotics if fever
Immunosuppressive therapy
▪ Indicated for patients > 50 years ▪ Patients with no HLA matched sibling
▪ A descriptive term for the clinical manifestation of haemolysis and haemoglobinuria manifest by dark coloured urine in the morning.
▪ Paroxysmal nocturnal haemoglobinuria.
Moderate or severe hypocellularity found at least one aspiration (biopsy) site. Nonhaematopoietic cells increase in BM.
Diagnosis
Other diseases which may cause decreased blood count are carefully excluded，such as myelodysplastic syndrome(MDS), acute myelogenous leukemia(AML).
Treatment
Supportive Care
Growth Hormones
Immune Suppressive
Therapy
Hematopoietic Stem Cell
Transplantation
Supportive Care
▪ Red cell transfusion ▪ Platelet transfusion ▪ Management of neutropenia
Genetic aspects: HLA associated susceptibility
Seeds Soil
Harmful Insects
Presenting symptoms
Acute AA
Chronic AA
Onset acute, short history Anemia severe, remarkable
Normal BM (high power magnification)
Note the presence of megakaryocytes, erythroid islands, and granulocytic precursors. This marrow is taken from the posterior iliac crest in a middle aged person, so it is about 50% cellular, with steatocytes admixed with the marrow elements.
HSCT-HLA identical sibling HSCT
▪ Age <55 years. ▪ Conditioning with Cyclophosphamide &
antithymocyte globulin, with cyclosporin and methotrexate. ▪ Long term overall survival = 80-90% ▪ Chronic graft versus host disease (GVHD) remains a problem for 25-40% of patients.
slowly onset, longer history
relatively moderate
Bleeding heavy bleeding, multiorgan
Infection more frequent, sepsis
hemorrhage moderate, skin and mucus
moderate, upper respiratory infection
Incidence
China Annual incidence
7.4 per 100,000
1.4 6.0
Acute Chronic
Epidemiology
Distribution
female
male
ቤተ መጻሕፍቲ ባይዱ
Infant
70’s
Etiology
➢Chemical factors: drug (anticarcinogen, chloramphenicol,