Chronic Leukemia
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Chronic leukemia
The first affiliated hospital of Zhengzhou University Xie Xinsheng
12/01/09
1
Classification
12/01/09
Chronic myelogenous leukemia(CML) Chronic lymphocytic leukemia(CLL) 2
CML 12/01/09
3
Definition and clinical characters
12/01/09
Chronic myelogenous leukemia(CML) is a hemopoietic stem cell disease Granulocytosis splenomegaly Philadelphia(Ph) chromosome ABL-BCR fusion gene 4
Clinical manifestation
Symptoms:
The symptoms are vague, nonspecific, and gradual in onset.including: easy fatigability, anorexia, abdominal discomfort and early satiety, weight loss, and excessive sweating.
12/01/09
5
Clinical manifestation
Symptoms: Hypermetabolism: night sweat, heat intolerance, weight loss Stupor from the leukostasis(Cerebral vessels, pneumoangiogram) Spontaneous rupture of the spleen
12/01/09
6
Clinical manifestation
Signs: Pallor Splenomegaly(be present in about 90% patients at diagnosis) Sternal tenderness.
12/01/09
7
Laboratory
12/01/09
Blood The total leukocyte count is always elevated(always over 25000/ul) granulocytes at all stages of development are present and normal in appearance the blast is usually about 3% hypersegmented neutrophils are commonly present
8
Laboratory
Blood
Red blood cells are slightly decreased in the early stage Platelet count is elevated in about 50% patients at diagnosis and normal in most of the rest.
12/01/09
9
Laboratory
12/01/09
Bone marrow Markedly hypercellar Granulopoiesis is dominant, G/E rate is raised. The blast is less than 10% Eosinophils and basophils may be increased. Erythropoiesis is usually decreased, and megakaryocytes are normal or increased in number.
10
Myeloblast promyelocyte myelocyte metamyelocyte stab cell segmented leukocyte
12/01/09
11
Laboratory
neutrophil alkaline phosphatase
The activity of NAP is decreased or negative. The activity will be normal if the therapy is utility, and the activity is also increased when the patient’s condition is in progression or when the patient is combined with infection or in pregnancy.
12/01/09
12
Laboratory
Cytogenetics Over 90% patients contain the Ph chromosome, t(9,22)
12/01/09
13
Laboratory
12/01/09
Uric acid: an increased production of uric acid with hyperuricemia and hyperuricosuria occurs in untreated CML The level of serum lactic dehydrogenase(LDH) is elevated Level of B12-binding capacity is increased 14
Diagnosis
12/01/09
Splenomegaly, hypermetabolism Elevated total leukocyte count, Eosinophils and basophils increase Marrow is hypercellar Ph chromosome Bcr/abl gene NAP(-)
15
Differential diagnosis
Reactive leukocytosis: usually these leukemoid reactions occur in the setting of an overt inflammatory disease, cancer, or an infection. NAP is strong positive, Ph chromosome (-)
12/01/09
16
Clinical phase Chronic phase Accelerated phase Blastic phase or blast crisis
12/01/09
17
Clinical phase
Accelerated phase
Febrile, progressing anemia or bleeding Bone ache Progressing splenomegaly The medicine originally utility is invalid now The Plt is increasing or decreasing for unknown reason The blast is >10% in the blood or marrow The basophils are >20% in the blood Chromosome abnormalities other than Ph chromosome
12/01/09
18
Clinical phase
12/01/09
Blastic phase or blast crisis The blast is >20%,or lymphoblast and prolymphocyte, monoblast and promonoblast >20% The myeloblast and promyelocyte >30% in the blood The myeloblast and promyelocyte>50% in the marrow The blast infiltration into other organs 19
Treatment
Chemotherapy
hydroxyurea 1-4g/d, orally, depending on the height of the white cell count, it should be adjusted individually to keep the WBC at about 10000/ul busulfan 4-6mg/d,orally, can be used until the WBC falls to 10000/ul low dose cytosine arabinoside 1530mg/m2 12/01/09
20
Treatment
interferon interferon-α3000000 to 9000000 units subcutaneously each day,produces a normalization of blood counts in about 3% of patients, about 35% of patients have a dcreased proportion of Ph chromosome
12/01/09
21
Treatment Imatinib mesylate alloHSCT Radiotherapy and splenectomy Leukaphersis
12/01/09
22
The first affiliated hospital of Zhengzhou University Xie Xinsheng
12/01/09
1
Classification
12/01/09
Chronic myelogenous leukemia(CML) Chronic lymphocytic leukemia(CLL) 2
CML 12/01/09
3
Definition and clinical characters
12/01/09
Chronic myelogenous leukemia(CML) is a hemopoietic stem cell disease Granulocytosis splenomegaly Philadelphia(Ph) chromosome ABL-BCR fusion gene 4
Clinical manifestation
Symptoms:
The symptoms are vague, nonspecific, and gradual in onset.including: easy fatigability, anorexia, abdominal discomfort and early satiety, weight loss, and excessive sweating.
12/01/09
5
Clinical manifestation
Symptoms: Hypermetabolism: night sweat, heat intolerance, weight loss Stupor from the leukostasis(Cerebral vessels, pneumoangiogram) Spontaneous rupture of the spleen
12/01/09
6
Clinical manifestation
Signs: Pallor Splenomegaly(be present in about 90% patients at diagnosis) Sternal tenderness.
12/01/09
7
Laboratory
12/01/09
Blood The total leukocyte count is always elevated(always over 25000/ul) granulocytes at all stages of development are present and normal in appearance the blast is usually about 3% hypersegmented neutrophils are commonly present
8
Laboratory
Blood
Red blood cells are slightly decreased in the early stage Platelet count is elevated in about 50% patients at diagnosis and normal in most of the rest.
12/01/09
9
Laboratory
12/01/09
Bone marrow Markedly hypercellar Granulopoiesis is dominant, G/E rate is raised. The blast is less than 10% Eosinophils and basophils may be increased. Erythropoiesis is usually decreased, and megakaryocytes are normal or increased in number.
10
Myeloblast promyelocyte myelocyte metamyelocyte stab cell segmented leukocyte
12/01/09
11
Laboratory
neutrophil alkaline phosphatase
The activity of NAP is decreased or negative. The activity will be normal if the therapy is utility, and the activity is also increased when the patient’s condition is in progression or when the patient is combined with infection or in pregnancy.
12/01/09
12
Laboratory
Cytogenetics Over 90% patients contain the Ph chromosome, t(9,22)
12/01/09
13
Laboratory
12/01/09
Uric acid: an increased production of uric acid with hyperuricemia and hyperuricosuria occurs in untreated CML The level of serum lactic dehydrogenase(LDH) is elevated Level of B12-binding capacity is increased 14
Diagnosis
12/01/09
Splenomegaly, hypermetabolism Elevated total leukocyte count, Eosinophils and basophils increase Marrow is hypercellar Ph chromosome Bcr/abl gene NAP(-)
15
Differential diagnosis
Reactive leukocytosis: usually these leukemoid reactions occur in the setting of an overt inflammatory disease, cancer, or an infection. NAP is strong positive, Ph chromosome (-)
12/01/09
16
Clinical phase Chronic phase Accelerated phase Blastic phase or blast crisis
12/01/09
17
Clinical phase
Accelerated phase
Febrile, progressing anemia or bleeding Bone ache Progressing splenomegaly The medicine originally utility is invalid now The Plt is increasing or decreasing for unknown reason The blast is >10% in the blood or marrow The basophils are >20% in the blood Chromosome abnormalities other than Ph chromosome
12/01/09
18
Clinical phase
12/01/09
Blastic phase or blast crisis The blast is >20%,or lymphoblast and prolymphocyte, monoblast and promonoblast >20% The myeloblast and promyelocyte >30% in the blood The myeloblast and promyelocyte>50% in the marrow The blast infiltration into other organs 19
Treatment
Chemotherapy
hydroxyurea 1-4g/d, orally, depending on the height of the white cell count, it should be adjusted individually to keep the WBC at about 10000/ul busulfan 4-6mg/d,orally, can be used until the WBC falls to 10000/ul low dose cytosine arabinoside 1530mg/m2 12/01/09
20
Treatment
interferon interferon-α3000000 to 9000000 units subcutaneously each day,produces a normalization of blood counts in about 3% of patients, about 35% of patients have a dcreased proportion of Ph chromosome
12/01/09
21
Treatment Imatinib mesylate alloHSCT Radiotherapy and splenectomy Leukaphersis
12/01/09
22
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