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Hematopoiesis and disorders (Hematopoietic stem cells (Embryonic and fetal…
Hematopoiesis and disorders
Hematopoietic stem cells
CD34
Self-renewal
Self differentiation
Increased and occur in peripheral blood with administration of growth factor or chemotherapeutic agents
Embryonic and fetal
First month
Yolksac (blood islands)
6 weeks
Liver
Middle part of fetal life (4-5 month)
Liver (major), Spleen and lymph node (minor)
Latter half and post natal
Bone marrow
Growth factors
Erythropoietin
Erythrocytes
Thrombopoietin
Megakaryocytes (Plt,Thrombocytes)
G-CSF
Granulocyte
Neutrophil, Eosinophil, Basophil
Aplastic anemia (AA)
Classification
Acquired
Secondary
รู้ cause รักษาได้
Irradiation
Drugs
Infection
Idiopathic
Inherited
Fanconi anemia
Dyskeratosis congenita
Shwachman-diamon syndrome
Clinical presentations
Bleeding
Petechiae
Gum or nose bleeding
Heavy menstrual flow
Anemia
Fatigue
Shortness of breath
:red_cross: Do not have
:warning: Weight loss
:warning: Cachexia
:warning: Splenomegaly
:warning: Lymphadenopathy
Lab
Pancytopenia
RBC
Normochromic normocytic or macrocytic anemia
Low reticulocyte
WBC
Low WBC with :explode: Relative lymphocytosis (lymphocyte กลับมีสัดส่วนมากที่สุดแทน neutrophil) :!!:
Platelet
Thrombocytopenia
Bone marrow study
For confirm diagnosis
Aspiration and biopsy
Severe aplasia, hypocelluar bone marrow
Chromosome analysis
Usually normal
Treatment
Supportive
Bleeding
Plt transfusion
Anemia
RBC transfusion
Keep Hb > 7 or > หรือเท่ากับ 9 g/dl in CVS underlying
Definitive
Hematopoietic stem cell transplant
Immunosuppression
Paroxysmal nocturnal hemoglobinuria (PNH)
Definition
Acquired clonal somatic mutation of hematopoietic stem cell
PIG-A gene
สร้าง GPI-anchor protein
ให้ cell suface GPI-AP มาจับ ป้องกัน RBC แตก
CD59
Block C9 (เจาะรู)
CD55
Destruct C3 cinvertase
Classification
Classical
Chronic intravascular hemolysis with recurrent
Weakness, dyspnea, pallor
Hemoglobinuria ฉี่สีโค้กในตอนเช้า
Acute renal failure
Iron def. anemia
Thrombosis in unusual sites
Liver, spleen
Hypoplastic
Pancytopenia
Related AA disease (history of AA)
Diagnosis
Flow cytometry
Anti-CD59
Treatment
Supportive
Iron supplement
Blood transfusion
Immunosuppresive
Eculizumab
Against C5 (inhibit C activation
BM transplant