ANEMIA Flashcards
Anemia definition
Decrease below normal of RBCs, hemoglobin, or hematocrit.
Normocytic, Normochromic Anemia
MCV and MCHC are both normal.
Normocytic, normochromic Conditions with normal or decreased Reticulocyte Count
Aplastic anemia, Kidney disease, Acute blood loss.
Normocytic, normochromic Conditions with increased Reticulocyte Count
(ENZYMES and Hemolytic anemias)
PNH, Paroxysmal Cold Hemoglobinuria, Sickle Cell Disease, Enzyme deficiencies: G6PD deficiency, PK deficiency, Other hemolytic anemias.
Rare, potentially deadly bone marrow failure syndrome
Aplastic anemia.
Aplastic anemia features
Pancytopenia (↓ RBCs, WBCs, platelets), Reticulocytopenia, Bone marrow hypocellularity, Depletion of hematopoietic stem cells.
Paroxysmal Cold Hemoglobinuria
Autoimmune hemolytic anemia with Donath Landsteiner antibody (autoanti-P).
Approximately 80 to 85% of aplastic anemia cases
Acquired Aplastic Anemia.
Acquired Aplastic Anemia categories
1) Idiopathic acquired aplastic anemia (no known cause), 2) Secondary acquired aplastic anemia (associated with identified causes like chemicals, viruses, and drugs).
Common causes of Secondary acquired aplastic anemia
Chemicals (e.g., insecticides, benzene), Viruses (e.g., Epstein-Barr virus), Drugs (e.g., chloramphenicol).
Drug most frequently implicated in acquired aplastic anemia
Chloramphenicol.
Approximately 15% to 20% of aplastic anemia cases
Inherited Aplastic Anemia.
Inherited Aplastic Anemia diseases
1) Dyskeratosis congenita, 2) Shwachman-Bodian-Diamond syndrome, 3) Fanconi Anemia (FA).
Most common inherited aplastic anemia, a chromosome instability disorder
Fanconi Anemia (FA).
Fanconi Anemia features
Aplastic anemia, cancer susceptibility, and physical abnormalities (e.g., skeletal abnormalities, skin pigmentation changes, short stature, abnormalities of the eyes, kidneys, and genitals).
Fanconi Anemia physical abnormalities
Skeletal abnormalities (thumb malformations, microcephaly, scoliosis), Skin pigmentation changes (hyperpigmentation, hypopigmentation, café-au-lait lesions), Short stature.
Fanconi Anemia vs Fanconi Syndrome
Fanconi Syndrome is a defect in proximal CT.
Paroxysmal Nocturnal Hemoglobinuria (PNH) A.K.A
Marchiafava-Micheli Syndrome.
Paroxysmal Nocturnal Hemoglobinuria (PNH) cause
Deficiency of complement regulatory proteins: DAF (decay-accelerating factor, CD55), MIRL (membrane inhibitor of reactive lysis, CD59).
Test for PNH
Ham’s Acidified Serum Test, Sugar Water Test (sucrose hemolysis test); Flow cytometry using FLAER (confirmatory for PNH).
FLAER in PNH
Fluorescein labelled proacrolysin variants.
Microcytic, Hypochromic Anemias
Small cells with increased central pallor (MCV and MCHC both LOW).
Microcytic anemia cause
Insufficient iron for normal erythropoiesis.
Microcytic anemia diagnosis
Abnormal iron studies.
Early microcytic anemia development
Reduced iron stores, no obvious anemia.
Associated conditions of microcytic hypochromic anemia (C T A I L S)
(Chronic blood loss, Thalassemia, Anemia of chronic inflammation, Iron deficiency anemia, Lead poisoning, Sideroblastic anemia).
Anemia of chronic inflammation (ACI) original name
Anemia of chronic disease (ACD).
Most common anemia among hospitalized patients
Anemia of chronic inflammation (ACI).
Conditions associated with ACI
Chronic infections (e.g., tuberculosis), Chronic inflammatory conditions (e.g., rheumatoid arthritis), Tumors.
Central feature of ACI
Sideropenia (↓ serum iron) despite abundant iron stores.
PBS in ACI
Usually normocytic, normochromic; advanced stages: microcytic, hypochromic.
Acute phase reactants (APRs) in ACI
Hepcidin, Ferritin, Lactoferrin.
Hepcidin function
Master regulatory hormone for systemic iron metabolism; inactivates ferroportin (iron transport from tissues to blood).
Sideroblastic anemia development
Prevented protoporphyrin production or the incorporation of iron into protoporphyrin.
Sideroblast definition
Nucleated RBC precursor with cytoplasmic iron granules.
Siderocyte definition
Anucleate RBC with iron granules.
Iron in sideroblastic anemia
Trapped in mitochondria, cannot be fully utilized in Hb synthesis.