Hematology exam review (summer) Flashcards
Hgb number in anemia?
Male <12
Female <15
Where is iron absorbed?
Duodenum
What conditions is iron absorbed?
Acidic conditions
Which iron study reflects total body stores of iron? Normal value?
Serum Fe
>30 is normal
Men and non-menstural women need how much iron/day?
1mg/day
Menstural women need how much iron/day?
3-4mg/d
Preggers women need how much iron/day?
2-5mg/d
Normal Recitulocyte Count range?
0.5-2%
What does Reticulocyte Count measure?
Bone marrow function.
High Reticulocyte Count means what type of cells being produced?
Immature cells
Normal Reticulocyte Index Count range?
2-3
Normal Serum B12? Deficiency?
Normal >210
Deficiency <170
Normal RBC Folic Acid level? Deficiency?
Normal >140
Deficiency <140
What is #1 MC anemia?
Iron Deficiency Anemia
MC cause of Iron Deficiency Anemia?
BLEEDING (menstural or GI)
How often screen 12-18 y/o F (non-preggers) for Iron Deficiency Anemia?
q5-10 year
When to first screen preggers for Iron Deficiency Anemia?
At first prenatal visit
When to screen men and post-menopausal F for Iron Deficiency Anemia?
No screening
What very weird craving in Iron Deficiency Anemia?
Pagophagia (ice craving)
What happens to tongue, nails, and corners of mouth in severe Iron Deficiency Anemia?
Brittle nails, nail spooning (koilonychia), and cheilosis of mouth corners
How much iron lost from menstural bleed?
1mg/day
How much iron lost from preggers, delivery, and nursing?
1g/d
How much iron lost per 100mL whole blood?
50mg Fe per 100mL whole blood
CBC and Iron Deficiency Anemia?
Hgb low, RBC low
TIBC up or down in Iron Deficiency Anemia?
Up. More binding places for iron due to not enough iron.
Serum Fe, Serum Iron, and % Sat in Iron Deficiency Anemia?
DOWN
Two essential lab tests for Iron Deficiency Anemia?
Hgb <12 or 15, Serum Fe <30
MCV and MCHC levels in Iron Deficiency Anemia?
Microcytic <80
Hypochromic <27
TOC for Iron Deficiency Anemia?
Ferrous Sulfate 325mg TID x1-2 mo until normal CBC
When to do CBCs in Iron Deficiency Anemia?
3 weeks (half better), 2 months (normal), 6 months (normal and stores rebuilt)
Tx of Iron Deficiency Anemia months 3-6?
325mg PO qd to rebuild stores
2 things which increase iron absorption?
- Empty stomach
2. Orange juice
SEs of Ferrous Sulfate?
Nausea, constipation, diarrhea, dark stool. Reduce dose.
When to use IV iron in Iron Deficiency Anemia?
Can’t absorb iron orally. IBD, gastric bypass, etc.
What is reduced in Thalassemia?
Reduced production of alpha or beta blobin chains
Alpha Thalassemia reduction of what?
Reduced alpha globin production
Who gets Alpha-1-Thalassemia? (two)
MC in S.E. Asia and China
Geneotype in Alpha-1-Thalassemia?
Heterozygous. Each parent missing one gene.
alpha ____/alpha ____
Who gets Alpha-2-Thalassemia?
MC black
Geneotype in Alpha-2-Thalassemia?
Homozygous. One parent missing each.
alpha alpha/____ ____
Genotype in Alpha Thalassemia Intermedia (aka Hgb H Dz)?
alpha ___/___ ___
Genotype of Alpha Thalassemia Majora (aka Hydrops Fetalis)?
No alpha globin at all
___ ___ / ___ ___
MCV in Alpha Thalassemia?
Microcytic
What does electrophoresis show in Alpha Thalassemia trait?
Normal Hgb.
What does electrophoresis show in Alpha Thalassemia Intermediate (Hgb H Dz)?
10-40% HgbH
S/Sx of Alpha Thalassemia trait?
No clinical S/Sx.
Tx of Alpha Thalassemia trait? Life expectency?
No tx. Normal expectency.
S/Sx of Alpha Thalassemia Intermediate (Hgb H Dz)?
Pallor, splenomegaly, fatigue, weakness
TIBC normal/increased/decreased in Alpha Thalassemia?
Normal or increased
Iron normal/increased/decreased in Alpha Thalassemia?
Normal or increased
Tx for Alpha Thalassemia Intermediate (Hgb H Dz)?
Folic Acid 1mg PO
What to avoid with Alpha Thalassemia Intermediate (Hgb H Dz)?
Avoid oxidative (sulfa) drugs, avoid iron
When to tranfuse PRBC in Alpha Thalassemia Intermediate (Hgb H Dz)? Goal Hgb?
If severely low Hgb. Goal 9-10.
Tx for refractory Alpha Thalassemia Intermediate (Hgb H Dz)? (Hint: take something out)
Splenectomy
When needed if getting regular transfusions for Alpha Thalassemia?
Iron chelation with Deferastrox 20-30mg/kg/d
Type of counseling needed for Alpha Thalassemia?
Genetic
Genetic bases of Beta Thalassemia?
Point delection on chromosome 11
Which globin chains are affected in Beta Thalassemia?
Beta-globin chains
What happens to Beta-globin chains in Beta Thalassemia?
Replaced with Beta-like chains (Delta and Gamma)
Beta Thalassemia MC in which population? (Hint: 2)
Mediterranian and Middle Eastern
Genotype of Beta Thalassemia Minor (aka Trait)? (Hint: two)
B/B+ or B/B˚
What percent are Beta-chains reduced in Beta Thalassemia Minor/Trait?
50%
S/Sx of Beta Thalassemia Minor/Trait?
ASx
CBC shows what in Beta Thalassemia Minor/Trait?
Clinically insignificant microcytic anemia
Electrophoresis shows what in Beta Thalassemia Minor/Trait?
HgbA2 4-8%
Tx required in Beta Thalassemia Minor/Trait?
No tx required
Counseling in Beta Thalassemia Minor/Trait?
Genetic counseling
Genotype of Beta Thalassemia Intermedia?
B+/B+
homozyous
When does Beta Thalassemia Intermedia begin to manifest?
6mo old when HgbF goes away
Where are bone deformities in Beta Thalassemia Intermedia?
Face
What type of fractures in Beta Thalassemia Intermedia?
Pathologic
Spleen and liver in Beta Thalassemia Intermedia?
Hepatosplenomegaly
When does severe anemia start in Beta Thalassemia Intermedia?
6 months
Three types of hemoglobin in Beta Thalassemia Intermedia?
HgbA 30%
HgbA2 10%
HgbF 6-100%
What does Peripheral Smear show in Beta Thalassemia Intermedia?
Bsophillic Stipling
Beta Thalassemia Majora aka?
Cooley Anemia
Growth and deformity in Beta Thalassemia Majora/Cooley Anemia?
Stunted growth, bone deformity
Spleen and liver in Beta Thalassemia Majora/Cooley Anemia?
Hepatosplenomegaly and jaundice
MCV in Beta Thalassemia Majora/Cooley Anemia?
Severe low
Majority of Hgb type in Beta Thalassemia Majora/Cooley Anemia?
HgbF
Tx of choice in Beta Thalassemia Majora/Cooley Anemia?
Allogeneic stem cell transplant
How often to transfuse PRBC in Beta Thalassemia Majora/Cooley Anemia?
Weekly
What to avoid in Beta Thalassemia Majora/Cooley Anemia?
Oxidative meds (sulfa, iron)
How to treat iron overload?
Deferoxamine
Prophyria due to what?
No porpherin ring in RBC
Skin in Prophyria?
Very pale skin
Light exposure does what in Prophyria?
Burns, blisters
Teeth in Prophyria?
Vampire-like teeth
Anemia of Chronic Dz due to what?
Conditions which reduce iron uptake
EPO response in Anemia of Chronic Dz?
Down
S/Sx in Anemia of Chronic Dz?
Fatigue, weak, dizzy
Hepcidin levels in Anemia of Chronic Inflammation?
Increased!
Serum Iron levels in Anemia of Chronic Inflammation?
Low
Serum Fe levels in Anemia of Chronic Inflammation?
Normal or inc
RBC life and mass in Anemia of Chronic Dz?
Decreased
Tx for Anemia of Chronic Dz?
Tx underlying condition
Folic Acid and/or Iron def
When to transfuse PRBC in Anemia of Chronic Dz?
If severe anemia
What med to give in Hgb <10 in Anemia of Chronic Dz? Goal Hgb?
Recombinant EPO until 10-12
Shape of Sideroblast in Sideroblastic Anemia?
Ringed sideroblast.
Where is ringed sideroblast found in Sideroblastic Anemia?
Bone Marrow
Iron levels in Sideroblastic Anemia?
Too much iron. Can’t get into RBC.
4 causes of acquired Sideroblastic Anemia?
- Cu deficiency
- INH
- Linezolid
- Chloramphenicol
Tx for INH acquired Sideroblastic Anemia?
B6 to fix
TIBC in Sideroblastic Anemia?
DOWN
SI, SFe, and % Sat in Sideroblastic Anemia?
Up!
Dx for Sideroblastic Anemia?
Bone marrow biopsy
Why splenectomy in congenital Sideroblastic Anemia?
Tertiary RBC production center.
Lead Toxcity does what to iron binding?
Decreases iron binding in proporphyrin ring
Chromic in Lead Toxcity?
Hypochromic
What type of cells seen on peripheral smear in Lead Toxcity?
Basophillic Stipiling
Basophillic Stipiling seen in what type of toxicity?
Lead Toxcity
Tx for Lead Toxcity?
Chelate
MCV in Alcoholic Anemia?
Macrocytic 100-110
How much EtOH per day to cause Alcoholic Anemia?
80g/day (1 bottle of wine)
Tx for Alcoholic Anemia? When resolves?
Stop drinking. Resolves in 2-4 months.
Myelodysplastic Syndromes are due to an inappropriately low response to what?
Reticulocytes
Which population has 2-3x higher instance of Aplastic Anemia?
East Asian
Ages of Aplastic Anemia?
Biphasic. 10-25, >60.
Aplastic Anemia due to injury of what?
Pluripotent stem cells in BM. Impairs proliferation and differentiation.
What is low in Aplastic Anemia?
Low PLT, RBCs, WBCs
Causes of Aplastic Anemia?
Viruses, drugs, chemo, radiation, toxins
What does CBC show in Aplastic Anemia?
Pancytoanemia
What does Peripheral Smear show in Aplastic Anemia?
Look normal but reduced number
Reticulocyte Count/Index increased or decreased in Aplastic Anemia?
Decreased
Tx for Aplastic Anemia under 20 y/o?
Allogenic hepatopoietic stemm cell transplant
Tx for Aplastic Anemia under 50 y/o?
Immunisuppressive therapy
Most common type of Aplastic Anemia?
Fanconi Anemia
Fingers and Fanconi Anemia?
Sindactyly (fused digits)
Where is B12 absorbed?
Terminal Ileum
What is B12 a cofactor for synthesis of? (Hint: 2)
- Nucleic acid synthesis
2. Myelin synthesis
How long are B12 stores good for?
3 years
Causes of B12 anemia?
Vegans, Pernicious Anemia, Chron’s DZ, EtOH
Pernicious Anemia what type immune disease?
Autoimmune.
What is destroyed in Pernicious Anemia by Abs?
Parietal cells and intrinsic factor
MCV in Pernicious Anemia?
>
- Megaloblastic anemia.
Blood smear in Pernicious Anemia?
Hypersegmented neurophils and macro-ovalcytes.