Hematology Flashcards
megaloblastic anemias
Macrocytic-Normochromic Anemias
Pernicious anemia
folate deficiency anemia
Methylmalonic acid and homocysteine levels are elevated early in the disease
pernicious anemia evaluation
Folate (folic acid) deficiency anemia s/s
Severe cheilosis: Scales and fissures of the lips and corners of the mouth
Stomatitis: Mouth inflammation
Painful ulcerations of the buccal mucosa and tongue: Characteristic of burning mouth syndrome
Dysphagia (difficulty swallowing), flatulence, and watery diarrhea
Folate (folic acid) deficiency anemia and CNS s/s
usually not seen
Pernicious anemia s/s
Weakness, fatigue
Paresthesias of the feet and fingers, difficulty walking
Loss of appetite, abdominal pains, weight loss
Sore tongue that is smooth and beefy red, secondary to atrophic glossitis
“Lemon yellow” (sallow) skin as a result of a combination of pallor and icterus
Neurologic symptoms from nerve demyelination - Not reversible
- Most common type of anemia worldwide
Iron-deficiency anemia
Sideroblastic anemia cause
defect in mitochondrial heme synthesis. Altered mitochondrial metabolism causes ineffective iron uptake and results in dysfunctional hemoglobin synthesis
o Sideroblasts
Erythroblasts contain iron granules that have not been synthesized into hemoglobin.
- Reversible sideroblastic anemia a/w
Associated with alcoholism
Sideroblastic anemia s/s
o Iron overload (hemochromatosis)
o Enlarged spleen (splenomegaly) and liver (hepatomegaly)
Sideroblastic anemia diagnostic
o Dimorphism
o Dimorphism
Normocytic and normochromic cells concomitantly observed with microcytic-hypochromic cells
Sideroblastic anemia tx (hereditary)
Pyridoxine (B6) therapy; life-long maintenance therapy at a lowered dose
Sideroblastic anemia tx (congenital)
Stem cell transplantation
Sideroblastic anemia tx (myelodysplastic synd (decreased RBC production))
Recombinant human erythropoietin
- Pancytopenia
Reduction or absence of all three types of blood cells
- Most aplastic anemias are
autoimmune disorders
Aplastic anemia patho
Hypocellular bone marrow that has been replaced with fat
- Paroxysmal nocturnal hemoglobinuria: deficiency in
CD55 and CD59: Cause complement-mediated intravascular lysis and release of hemoglobin
hapten model
Drug-induced hemolytic anemia
Drug-induced hemolytic anemia most common cause
cephalosporins. others also PCN and hydrocortisone
hemolytic anemia s/s
o May be asymptomatic
o Jaundice (icterus)
o Splenomegaly
hemolytic anemia tx first line
Corticosteroids
hemolytic anemia tx 2nd line
Splenectomy and Rituximab (Rituxan) (monoclonal antibody)
o Paroxysmal nocturnal hemoglobinuria tx
Eculizumab (Solaris)
- Polycythemia vera: acquired mutation in
Janus kinase 2 (JAK2) - * Negates the self-regulatory activity of JAK2 that allows the erythropoietin receptor to be constitutively active, regardless of the level of erythropoietin.
Polycythemia vera can convert to
acute myeloid leukemia (AML)
Polycythemia vera s/s
splenomegaly. painful itching by heat or water
Polycythemia vera tx
phlebotomy )300-500cc). spirin, interferon-a, hydroxyurea, radioactive phosphorus
- Hereditary hemochromatosis: autosomal…
recessive
Hereditary hemochromatosis
increased gastrointestinal iron absorption with subsequent tissue iron deposition
Hereditary hemochromatosis s/s
Hepatomegaly, abnormal liver enzymes, bronzed skin, diabetes, and cardiomegaly
Hereditary hemochromatosis tx
Phlebotomy; refrain from taking iron and vitamin C supplements and consuming raw shellfish; alcohol use in moderation
AML mutation
tyrosine kinase FLT3
ALL genetic anomoly
Philadelphia chromosome on chromosome 9and 22
CML genetics
Philly chromosome present and BCR-ABL1 causes intiaiton
CLL most common s/s
lymphadenopathy
CML cure
no cure
non hodgkins lymphoma cells
B cells, T cells, and NK cells
more localized: hodgkins or non?
hodgkins
Burkitt lymphoma
highly aggressive B-cell non-hodgkin lymphoma of jaw. EBV 90%. abd swelling. Rituximab tx
lymphoblastic lymphoma
rare non-hodgkin type.
lymphoblastic lymphoma population
1/3 in male children and adolescents
lymphoblastic lymphoma origin
T-cell in thymus 85%
lymphoblastic lymphoma first s/s
painless lymphadenopathy in neck
multiple myeloma translocation
imglobulin heavy chain on chromo 14
multiple myeloma patho
production of too much M protein (abnorm antibody). Light chains (Bence Jones proteins) pass through glomerulus and cause kidney damage
multiple myeloma s/s
hypercalc, renal failure, anemia, lytic lesions, infections