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
Besides hemolytic anemia, what else is seen in Cold Agglutinin immunohemolytic anemia?
Vascular obstruction in cold extremities –> pallor, cyanosis, Raynaud phenomenon
Cold Hemolysis immunohemolytic anemia - cause?
Antibody type? Against what?
Child after viral infection
IgG - binds to P blood group
Cold Hemolysis immunohemolytic anemia - what happens?
Viral infection causes IgG autoantibodies –> RBCs circulate to COLD areas, IgG binds –> circulate to WARM areas, IgG causes complement-mediated lysis
Causes of trauma to RBCs that lead to hemolytic anemia
- Prosthetic cardiac valves
- Microangiopathies (DIC, TTP, HUS, malignant HTN, SLE, cancer)
Schistocytes, burr cells, helmet cells, triangle cells
Trauma-induced hemolytic anemia
Abnormally large erythroid precursors (promegaloblasts), giant metamyelocytes, and band forms in the BM
Megaloblastic anemia
Abnormally large RBCs and hypersegmented neutrophils in peripheral blood
Megaloblastic anemia
Vegans and vegetarians (B12 or folate)
B12 deficiency
Fish tapeworm (B12 or folate)
B12 deficiency
Chronic alcoholic (B12 or folate)
Folate deficiency
Pregnancy, infancy, hemolytic anemia (B12 or folate)
Folate deficiency
Hemodialysis (B12 or folate)
Folate deficiency
Oral contraceptives (B12 or folate)
Folate deficiency
Why the neuro symptoms in B12 deficiency?
Increased methylmalonate –> abnormal fatty acid accumulation in neuron lipids –> myelin breakdown
Initiating event to autoimmune gastritis and pernicious anemia
Autoreactive T-cell response against gastric mucosa, causing autoantibody formation against intrinsic factor
Pernicious anemia is associated w/ what else?
Thyroiditis, adrenalitis
Non-pernicious causes of B12 deficiency
- Achlorhydia (ex. patient taking HCl supplement)
- Gastrectomy
- Exocrine pancreas loss (can’t split B12-haptocorrin)
- Ileal resection or disease
- Fish tapeworm
- Pregnancy, hyperthyroid, cancer, chronic infection (all increased requirement)
Intestinalization, beefy glazed tongue, decreased HCl production, spastic paraparesis, paresthesias
Pernicious anemia
Pernicious anemia…what to be cautious of?
Gastric carcinoma and atherosclerosis risks
Elevated homocysteine, normal methylmalonate
Folate deficiency
TMPRSS6 mutation
Elevated hepcidin –> microcytic iron-deficient anemia
A patient is iron deficient, but has no dietary issues, no GI issues, no bleeding issues, and seems perfectly healthy. She does not respond to iron supplementation. Potential explanation?
TMPRSS6 mutation
Abnormally low hepcidin…explanation?
Hemochromatosis (primary or secondary)
Secondary hemochromatosis…major cause?
Ineffective hematopoiesis –> decreased hepcidin
Decreased transferrin saturation, decreased serum ferritin
Iron deficiency
Spooning of nails, hair loss, malabsorption, atrophic tongue
Iron deficiency
Patient craving ice chips, soil, clay, or paper
Pica - iron deficiency
Smooth glazed tongue, trouble swallowing foods, microcytic anemia
Plummer-Vinson syndrome
Increased PIBC
Iron deficiency
Low serum iron, reduced TIBC, increased hemosiderin
Anemia of chronic disease
Describe anemia of chronic disease
Patient has source of chronic systemic inflammation, causing increased IL-6, which causes increased hepcidin, which causes iron-starved erythroblasts despite having plenty in marrow macrophages. Hepcidin also inhibits EPO production.
Causes of ACD
- Osteomyelitis, bacterial endocarditis, lung abscess
- RA, regional enteritis
- Carcinomas, Hodgkins lymphoma
Baby with pancytopenia, kidney hypoplasia, spleen and bone anomalies…what is it? Cause?
Fanconi anemia - defective DNA repair
Causes of aplastic anemia
Chemo, benzene, chloramphenicol, gold salts, viral infections, non-hepatitis hepatitis, irradiation, other
How does chemo, radiation, etc. cause aplastic anemia?
Antigenic alteration of HSCs by the agent –> T-HELPER CELL suppression/destruction of HSCs via IFN-GAMMA and TNF
Anemia, decreased Hb and Hct, normal WBCs and platelets, lack of erythroid progenitors in the BM
Associated with what?
Pure red cell aplasia
Thymoma, LGL leukemia, drugs, autoimmune disorders, parvovirus (aplastic crises)
Myelophthisic anemia
Most common causes? (3)
BM failure due to space-occupying lesions that replace normal marrow components
Metastatic carcinomas, granulomatous disease, MPD spent phase
Leukoerythroblastosis, dacrylocytic cells
Marrow replacement of some kind
Effect of uremia on RBCs
Diminished EPO synthesis –> anemia
Hypothyroidism + anemia…what kind?
Normochromic, normocytic
Hepatocellular liver disease + anemia…why?
Lipid abnormalities –> RBC membranes acquire phospholipid and cholesterol –> destruction
