Hematologic Flashcards
What are symptoms of iron deficiency?
Pica, pagophagia
Koilonychia
Angular chelitis
Plummer-vinson (dysphagia + esophageal webs + atrophic gastritis + Fe deficiency)
How do you diagnose iron deficiency?
Fe < 150
CBC (microcytic, hypochromic):
Low MCV, MCHC, Fe, ferritin, & transferrin
Elevated RDW & TIBC
What are causes of iron deficiency?
MC = Blood loss (menstruation, occult - colon CA, hookworms)
Diet deficiency: pregnancy, breastfed infants
What are features of thalassemia?
Decreased production of globin chains
Microcytic w/ normal or increased Fe
What are the 4 types of alpha thalassemia? How many alleles are affected in each type?
- Silent carrier (1/4 alleles): asx
- Minor (2/4): mild microcytic anemia
- Intermedia aka Hgb H (3/4): chronic anemia, pallor, hepatosplenomegaly,frontal & maxilla bony overgrowth, gallstones, iron overload. Presents similar to beta-thal*
- Hydrops fetalis (4/4): stillbirth, Hgb Barts* (gamma tetramers)
How do you dx alpha thalassemia?
CBC:
Decreased MCV
Normal or increased RBC count
Normal or increased Fe
Peripheral smear:
Target cells*, teardrop cells, basophilic stippling
Heinz bodies in Hgb H
Hgb electrophoresis
How do you treat mild alpha thalassemia?
No tx needed
How do you treat moderate alpha thalassemia?
Folate (if retic count is high)
Avoid oxidative stress (s/a sulfa)
Avoid iron supplementation
How do you treat severe alpha thalassemia?
Weekly blood transfusions, vit C, folate
Iron chelating agents: IV Deferoxamine, PO deferasirox
Splenectomy (if refractory)
Allogeneic bone marrow transplant = definitive
Who is alpha thalassemia MC in? Who is beta thalassemia MC in?
Alpha: SE Asian
Beta: Mediterranean*, Africans
What are the types of beta thalassemia? How many alleles are affected in each type?
- Trait aka minor (1/2 alleles): asx or mild-mod anemia
- Intermedia: anemia, hepatosplenomegaly, bony disease
- Major aka Cooley’s anemia (2/2 alleles): become symptomatic at 6 mos, frontal bossing & maxillary overgrowth, hepatosplenomegaly, severe hemolytic anemia, gallstones, iron overload
How do you diagnose beta thalassemia?
CBC:
Decreased MCV
Normal or increased RBC count
Normal or increased Fe
Peripheral smear: target cells*, teardrop cells, basophilic stippling, nucleated RBCs
Hgb electrophoresis: HgbF increased, HgbA2 increased, HgbA decreased
Skull bossing on xray (“hair on end”)
How do you treat beta thal trait (minor)?
Offer genetic counseling
How do you treat beta thal major?
Periodic blood transfusions, vit C, folate
Avoid excess Fe
Iron chelating agents: IV deferoxamine, PO deferasirox
Splenectomy if refractory
Allogenic bone transplant = definitive
What vitamin increases the absorption of Fe absorption?
Vit C!
Where is B12 absorbed?
Terminal ileum
What causes B12 deficiency?
- Pernicious anemia: autoimmune destruction/loss of gastric parietal cells which secrete intrinsic factor
- Vegans
- Malabsorption: alcoholism , celiac, Crohn’s
- Decreased IF production (acid-reducing drugs, bypass surgery)
What are clinical manifestations of B12 deficiency?
Neurological sx: paresthesias, gait abnormalities, memory loss, dementia
GI sx
How do you diagnose B12 deficiency?
Peripheral smear: Increased MCV > 115
Increased homocysteine, increased methlmalonic acid
Pernicious anemia
What is acute lymphocytic leukemia?
Malignancy of lymphoid stem cells in bone marrow –> lymph nodes, spleen, liver
Who is ALL MC in?
Childhood, peak at 3-7yo
Down syndrome
What are the clinical manifestations of ALL?
Pancytopenia sx: fever MC, fatigue, lethargy, bone pain
CNS sx: HA, stiff neck, visual changes, vomiting
What does ALL look like on PE?
Pallor, fatigue
Petechiae, bruising
Hepatosplenomegaly, lymphadenopathy **
How do you diagnose ALL?
Bone marrow:
- hypercellular w/ > 20% blasts
- WBC 5-100,000
- anemia, thrombocytopenia
How do you treat ALL?
Oral chemo (hydroxyurea) Highly responsive to combo chemo
What is chronic lymphocytic leukemia?
B cell clonal malignancy
Who is CLL MC in?
Adults!
> 50 yo
Males
Caucasians
What are clinical manifestations of CLL?
Most asx
Fatigue MC, dyspnea on exertion
Increased infections
Lymphadenopathy, hepatosplenomegaly
How do you diagnose CLL?
Smear: well-differentiated lymphocytes w/ scattered “smudge cells”, lymphocytosis > 20,000
Pancytopenia: thrombocytopenia, anemia
How do you treat CLL?
Obs
Chemo (fludarabine)
Allogeneic stem cell transplant = cure
What is chronic myelogenous leukemia?
Well differentiated WBCs
Granulocyte proliferation