HEMATOLOGY

Primary Hemophagocytic Lymphohistiocytosis

Dec 5, 2017 by in HEMATOLOGY Comments Off on Primary Hemophagocytic Lymphohistiocytosis

Disease Defective gene Protein Inheritance Protein function FHL syndromes without hypopigmentation FHL2 PRF1 Perforin AR Pore formation FHL3 UNC13D Munc13-4 AR Vesicle priming FHL4 STX11 Syntaxin11 AR Vesicle fusion FHL5…

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Acute Lymphoblastic Leukemia

Dec 5, 2017 by in HEMATOLOGY Comments Off on Acute Lymphoblastic Leukemia

Study group Trial Years ALL subtypes No. of patients Age EFS, % (year) OS, % (year) Reference AIEOP/BFM AIEOP-BFM ALL 2000 2000–2006 B-ALL 4016 1–18 80.4 (7) 91.8 (7) Conter…

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Langerhans Cell Histiocytosis

Dec 5, 2017 by in HEMATOLOGY Comments Off on Langerhans Cell Histiocytosis

Disease Clinical characteristic Histologic features CD1a CD14 CD68 CD163 CD207 S100 Factor XIIIa Fascin HLA-DR Others Langerhans cell histiocytosis See text + − +/− − + + − − +…

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Pathogenesis and Treatment of Hemophilia

Dec 5, 2017 by in HEMATOLOGY Comments Off on Pathogenesis and Treatment of Hemophilia

Fig. 9.1 Linear representation of F8, the structure of FVIII, and its cleavage site. The 26 exons (above) and domain organization (below) of FVIII based on amino acid homology. Activation…

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Inherited Bone Marrow Failure Syndrome, TAM

Dec 5, 2017 by in HEMATOLOGY Comments Off on Inherited Bone Marrow Failure Syndrome, TAM

Fig. 7.1 Model of p53 stabilization in response to impaired ribosome biogenesis in DBA. Normal erythroblasts produce large numbers of ribosomes for protein synthesis. Levels of p53 remain low via…

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Acute Myeloid Leukemia

Dec 5, 2017 by in HEMATOLOGY Comments Off on Acute Myeloid Leukemia

AML with recurrent genetic abnormalities  AML with t(8;21)(q22;q22.1); RUNX1-RUNX1T1  AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22);CBFB-MYH11  APL with PML-RARA  AML with t(9;11)(p21.3;q23.3); MLLT3-KMT2A  AML with t(6;9)(p23;q34.1); DEK-NUP214  AML with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2);…

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Hemophagocytic Lymphohistiocytosis, Secondary

Dec 5, 2017 by in HEMATOLOGY Comments Off on Hemophagocytic Lymphohistiocytosis, Secondary

Fig. 13.1 Pathophysiology of infectious mononucleosis (a) and hemophagocytic lymphohistiocytosis (b) due to primary EB virus infection. EBV-IM EB virus-associated infectious mononucleosis, EBV-HLH EB virus-associated hemophagocytic lymphohistiocytosis, INFγ interferon, IL-6…

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Childhood Aplastic Anemia

Dec 5, 2017 by in HEMATOLOGY Comments Off on Childhood Aplastic Anemia

Idiopathic AA  Secondary AA Hepatitis associated Secondary (drug/chemical/radiation/infection) Inherited AA Fanconi anemia Dyskeratosis congenita Shwachman-Diamond syndrome Congenital amegakaryocytic thrombocytopenia Others Myelodysplastic syndrome Refractory cytopenia of childhood (provisional) Refractory cytopenia with…

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