Anemia - Ch. 14 Flashcards
Pale, weak, easily fatigued, exercise intolerance
Anemia
After 3-5 days…reticulocytosis, leukocytosis, thrombocytosis, erythroid precursor cells (BM) w/ blue-red cytoplasm, increased EPO
Anemia of acute blood loss
Anemia, splenomegaly, unconjugated jaundice, decreased haptoglobin
Extravascular hemolytic anemia
Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, unconjugated jaundice, red-brown urine, decreased haptoglobin
Intravascular hemolytic anemia
BM - normoblasts (erythroid precursors)
PB - reticulocytosis, hemosiderosis
Labs - increased EPO, increased degradation products
Hemolytic anemia
RUQ abdominal pain, hemolytic anemia
Indicate what?
What else tends to happen in this setting?
Pigment gallstones
CHRONIC hemolytic anemia
Splenomegaly
Caucasian, anemia, splenomegaly, jaundice, episode of worsened anemia symptoms during an infection
Hereditary spherocytosis - complicated by aplastic crisis (parvovirus infection)
Osmotic lysis in hypotonic salt solutions
Hereditary spherocytosis
Heinz bodies, bite cells, spherocytes
G6PD deficiency (the Heinz bodies cause the formation of bite cells and spherocytes)
Episodic anemia, jaundice, weakness, pallor. Black or Mediterranean male. No splenomegaly or gallstones.
G6PD deficiency
Caucasian teenager, anemia, splenomegaly, jaundice, episode of cervical lymphadenopathy and swollen tonsils w/ decreased haptoglobin
Hereditary spherocytosis - complicated by hemolytic crisis (infectious mononucleosis)
Sickle cell, rapid splenic enlargement, hypovolemia, shock
Sequestration crisis
Crewcut X-Ray, enlarged cheekbones
- Sickle cell disease
- Beta-thalassemia major
Beta-4 tetramers
Hemoglobin H - alpha-thalassemia - adolescents and adults
Gamma-4 tetramers
Hemoglobin Barts - alpha-thalassemia - young kids
Genetic differences in alpha vs beta thalassemias
Alpha - gene deletions
Beta - point mutations
Asians vs blacks - alpha-thalassemia genotypes
Asian - a/a -/-
Black - a/- a/-
HbH disease - explain
3 alpha-globin mutations = HbH formation = extreme tissue hypoxia, intracellular inclusions = red cell sequestration = moderate anemia
Hydrops fetalis - explain
Symptoms
4 beta-globin mutations = Hg barts formation + embryonic Hb tetramer formation
Pallor, genralized edema, massive HSM
Which thalassemias require regular blood transfusions?
Beta-major, hydrops fetalis
Intravascular hemolysis (anemia, jaundice, hemoglobinemia, hemoglobinuria, hemosiderin), autoimmune disease, venous thrombosis
Future consideration?
Paroxysmal nocturnal hemoglobinuria
Transform in AML or MDS (since its an HSC)
Immunohemolytic anemia - what is it?
Hemolysis type?
Premature destruction of RBCs due to antibodies or complement that attach
Extravascular (usually)
Causes of Warm Antibody Type immunohemolytic anemia
Antibody type? Against what?
- Idiopathic
- SLE
- Drugs - penicillin, cephalosporins, alpha-methyldopa
- Lymphoid neoplasms
IgG - anti-Rh
Warm Antibody immunohemolytic anemia - what happens?
IgG antibodies bind RBCs, causing (usually) extravascular hemolysis (loss of membrane –> spherocytes –> sequestration –> splenomegaly
Causes of Cold Agglutinin Type immunohemolytic anemia
Antibody type?
- Acute = Mycoplasma, EBV (mono)
- Chronic = idiopathic, lymphoid neoplasms
IgM (M = Mycoplasma)
Cold Agglutinin immunohemolytic anemia - what happens?
Child has self-limited infection OR chronic lymphoid neoplasm OR no identified cause –> RBCs circulate to COLD areas, IgM binds –> circulate to WARM areas, IgM releases –> C3b deposits cause extravascular hemolysis