Normo/Normo, Macro, & hemolytic Flashcards
What are the 4 causes of deficient erythropoieisis?
Renal Disease (any kidney damage)
Hypothyroidism (endocrine failure)
Aplastic Anemia
Myelophithisc
ACD can present as normo/normo as well as Micro/hypo
Decreased O2 need–> decreased ??? –> Decreased Basal Metabolism–> Decreased ???
EPO
RBC production
List causes of chronic kidney disease
Diabetes mellitus
Chronic Hypertension
Glomerulus Nephritis
Polycystic Kidney Disease
Recurrent Kidney Infection
Kidney excretory failure means it does not perform what two functions?
EPO secretion
Urine production
What do these symptoms indicate?
Edema & fluid build up
hypertension
fatigue
decrease urine output
increase in urination frequency
muscle cramping
Renal insufficiency
What will a CBC show of someone with kidney damage/renal insufficency?
Anemia
Normo/Normo
PBS= Echinocytes
What are the expected UA results of someone with renal damage?
Proteinuria
What additional tests can you run for someone that has expected kidney damage?
Kidney Function Test
Refer to Nephrologist for Renal Imaging
How can hypothyroidism cause a Normo/Normo anemia?
Decreased T4–> Decreased T3= decreased metabolism–> decreased O2 requirement–> decreased EPO output–> decreased RBC production
How can hypothyroidism cause a Hypo/Micro anemia?
Hypothyroid causes heavy menses–> chronic blood loss–> decreased iron ferritin–> decreased Iron serum–> IDA
How can hypothyroidism cause a Macrocytic anemia?
Decreased metabolism due to low T4 & T3–> decreased function of parietal cells–> decreased I.F. & decreased stomach acid–> decreased absorption of B12–> leads to decreased B12 deficiency= Macro.
What are the symptoms of Hypothyroidism? (7)
Fatigue
Brain fog
Increased sensitivity to cold
Constipation
Weight gain
Weakness
Thinning of out 1/3 of eyebrow
Since hypothyroidism can result in 3 different anemias what additional tests can be performed to diagnose it?
Thyroid Panel (determine function)
Iron panel
Serum B12 (can pair with a RBC folate test as well)
Patient with confirmed hypothyroidism has a macrocytic anemia from a B12 deficency. Best way to treat the patient?
Hormonal therapy. Correct the primary disease to treat the secondary disease as well.
What is the term for all of the tissue cells in the bone marrow has decreased production?
Panhypoplasia
(pancytopenia & hypocellular bone marrow)
What are the etiological causes of Aplastic Anemia?
Idiopathic
Drugs
Viral (immunocompromised)
Toxic exposure- glues, pesticides
Immune disorders
Congenital- Fanconi Anemia
Radiation treatment
What is the congenital cause of aplastic anemia called?
Fanconi Anemia- congenital failure of marrow
Which anemia has an increased susceptibilty to infections?
Aplastic anemia (decreased WBCs)
Aplastic anemia has an issue with bleeding tendencies due to?
Decreased platelets
What are the CBC findings of aplastic anemia?
Normo/Normo Anemia
Leukopenia of less than 1500 WBC
Neutropenia (all WBCs down)
Low reticulocyte count
Hypocellularity on bone marrow biopsy
Treatment for aplastic anemia?
Bone Marrow Transplant
(often refractory)
What are the 4 etiological causes of myelophthisic anemia?
Carcinoma metastasizing to bone marrow from primary tumor
Myelofibrosis
Myeloproliferative conditions
Osteopetrosis
What is myelofibrosis?
Polycythemia Vera elevating all cell lineages but leads to bone marrow replacement by scar tissue
What are 4 examples of myeloproliferative conditions?
Multiple myeloma, Leukemias, Lymphoma, Hodgkin’s
What condition results from increased bone deposition taking up bone marrow space?
Osteopetrosis
Myelophthisic anemia has 3 sets of symptoms. What are they?
Symptoms of Anemia
Symptoms of Underlying disease
Hepatomegaly & Splenomegaly
What lab findings indicate Myelophthisic anemia?
Normo/Normo anemia
Nucleated RBCs (due to increased production rate)
Reticulocytosis
Polychromatophilia (blue-ish RBCs)
Tear drop RBCs on PBS
What is the term for blue tinted RBCs?
Polychromatophilia
Why are the RBCs on a PBS for Myelophthisic anemia tear drop shaped?
Due to lack of space and squeezing the RBCs
What is significant about WBCs in a macrocytic anemia? What is this an early indicator for?
Hypersegmented Neutrophils; B9/B12 deficiency
What “bucket” does megaloblastic/Macrocytic anemias fit in?
Decreased production
How long is B12 stored in the liver?
5-6 years
What does B12 do to folic acid?
Reduces to Folate
B12 is important for?
Myelin synthesis
There are 3 systems that make tissues rapidly that are most affected by B12 deficiency. What are they?
GI tract
Hematopoetic system
Neuro system
How long could a B12 deficiency take to develop after a gastrectomy/gastric bypass? Why does this happen?
3-6 years due to liver storage.
Happens due to decreased intrinsic factor= less B12 absorbed
What are the 3 causes to deficient intrinsic factor?
Gastrectomy/Gastric Bypass
Myxedema (hypothyroidism)
Pernicious Anemia (Most Common)
What is Pernicious Anemia?
auto immune attack on intrinsic factor and/or parietal cells leading to B12 deficiency
What is Myxedema?
Severe Hypothyroidism
What does Myxedema cause?
Decreased in parietal cells & chief cells= decreased I.F. & stomach acid= less B12 absorption
What group should be supplementing B12 in their chosen diet?
Strict Vegans
What GI syndromes lead to B12 malabsorption syndromes?
Celiac Disease
Crohn’s disease
SIBO (small Intestinal Bacterial Overgrowth)
Chronic pancreatitis or pancreatic cancer
Why could you see discoloration of the skin (mild jaundice) in a B12 deficiency?
Megaloblast cells get destroyed at a higher rate.
Macrocytic blood cells are not as flexible/strong leading to increased free bilirubin
What are the 3 patient presentation categories for B12 deficiency anemia?
Hematopoetic
Gastrointestinal
Neurological
What are hematopoetic signs in patient presentation?
Anemia (any signs)
Discoloration of the skin
What are the gastrointestinal patient presenations for B12 deficency?
Glossitis- sore, “beefy red” tongue
Weight loss (digestive issues)
Anorexia (digestive complaint)
Constipation w/ diarrhea
Poorly localized abdominal pain
What is typically the 1st presentation & the MC complaint of B12 def?
Neurological- numbness/tingle in BIL hands & feet that moves proximal as it progresses
Can progress to DWC= loss of vibratory & position sense and ataxia
What do you expect to see on a neuro exam for B12 Def?
⬇️ position sense
⬇️ Vibratory sense
abnormal reflex
ataxia
(+) Babinski sign
extremity numbness
hypoalgesia “sock & glove”
List the 5 MC physical exam findings of B12 Def. anemia
Weight loss
Glossitis
Hepatomegaly/Splenomegaly
Skin pigmentation change (mild jaundice)
Anemia symptoms
CBC shows=
Anemia= Macrocytic
Anisocytosis= ⬆️ RDW
Mild Leukopenia
Mild Thrombocytopenia
PBS= Hyper-segmented neutrophils
What could this be?
B12 deficiency anemia
B9 deficiency anemia
What would the chem panel for B12 def. anemia look like?
⬆️ Serum bilirubin- Unconjugated (will NOT show up on UA)
⬆️ LDH isoenzyme 1 (found IN RBCs)
What special tests should you run to confirm B12 Deficiency anemia?
Serum B12
Follow up w/ Auto-antibody test for I.F. & Parietal Cell antibodies
MMA test- WNL B12 and ⬆️ MMA shows B12 is not being used by the body
What does the timeline for B12 Tx look like?
Hematologic correction- 6 weeks
Neural improvement- can take up to 18 months
Retest Serum B12 every 1-2 month post high dosing Tx. MMA as well to see it is being utilized
What is the precaution with folic acid Tx?
Folic acid treatment without B12 in patients who have pernicious anemia is contraindicated. Can cover up early symptoms and/or make neuro symptoms worse
What is the function of Folic acid?
Needed for synthesis of purines & pyrimidines aka DNA maturation
Where is B9 absrobed?
Duodenum & jejunum
What is the ONE test that will differentiate Folic Acid deficiency from B12 deficiency?
RBC Folate Level= Would be ⬇️ & serum B12 would be WNL
What groups are at risk for folate def?
Deficient dietary intake- “tea & toasters” or Chronic Alcoholics
Chronic Liver Disease
Increased requirements- Pregnancy & lactation
MC cause of B9 def?
Pregnancy & lactation
What are the symptoms of B9 def? What is the one symptom that B9 does NOT share with B12?
Anemia Sx’s
Gi disturbance
Does NOT have neuro deficits
This is your CBC panel=
Anemia= Macrocytic
Anisocytosis= ⬆️ RDW
Mild Leukopenia
Mild Thrombocytopenia
PBS= Hyper-segmented neutrophils, Macrocytes.
You think it is B12 or B9 deficiency. What tests are you using to decide between the two?
Serum B12
RBC folate
How are you treating B9 def?
Active Folate (5-MTHF)
400-1000 mg/day
1-3 months, then retest
400-800mcg/day for pregnancy through lactation
What does hemolytic anemia mean?
Shortened RBC lifespan,
hemolysis,
Bone marrow production can not keep up
What 3 clinical findings can occur with hemolytic anemias?
Jaundice
splenomegaly
hepatomegaly
What lab tests should be run for all hemolytic anemias?
LDH levels
Serum Unconjugated bilirubin
Serum Haptoglobin
Reticulocyte count
What are the 4 genetic categories of hemolysis due to intrinsic RBC defects?
Hereditary spherocytosis
G6PD defects
Sickle Cell anemia
Thalassemia
Congenital issue with the extracellular matrix of RBCs that results in it not being able to maintain a biconcave shape
Hereditary Spherocytosis
What is this?
CBC=
Mild/Moderate Anemia
Hb not below 8g/dL
MCHC ⬆️ (unique to this anemia)
PBS= Spherocytes
Hereditary Spherocytosis
What test is used to confirm Spherocytosis?
Osmotic Fragility Test= will be ⬆️
How do you manage Spherocytosis?
Blood Transfusions
Splenectomy
What does G6PD cause?
⬆️ Reactive Oxygen Species (⬆️ cell damage)
Heinz Bodies (darkened areas on RBC)
Bite Cells (macrophages attacking the Heinz Bodies)
Why is G6PD Def Anemia more common in males?
it is an X-linked abnormality
When does G6PD anemia start?
Normal life until “triggered”=
Certain drugs
Certain chemicals
Infections- Viral & bacterial
Stressors
FAVA BEANS
What are the clinical findings of G6PD anemia?
Any anemia, jaundice, & hepat/splenomegaly
Difficulty fighting infections due to neutrophil involvement
What does the following lab findings indicate?
CBC- Moderate anemia
PBS- Bite cells
G6PD anemia
What are the 2 types of Sickle Cell Anemia?
Homozygous (inherit 2 bad genes)= sickle cell anemia
Heterozygous (inherit 1 bad gene)= sickle cell trait
Why is Sickle Cell Trait not as serious as Sickle Cell Anemia?
SCT is usually asymptomatic w/o anemia. There is not enough of an abnormality in the Hb of SCT to create sickle cells so there is far less risk
What causes issues with Sickle Cell Trait?
Exertional Sickling=
caused by intense physical activity
dehydration
Low O2/High Alt
What causes sickle cell anemia?
Beta chains of Hb not synthesized correctly
What are the normal levels of Hb A, Hb F, & Hb A2?
HbA= 95-98% of Hb
HbF= 2-3%. Larger percentage until 6 months.
HbA2= 0-1%
What chains make up each Hb?
HbA= 2 alphas, 2 Betas
HbF= 2 alphas, 2 Gammas
HbA2= 2 alphas, 2 deltas
Why is the substitution for Valine in place of Glutamic Acid what causes Sickle Cell Hb and the sickle shape?
Valine is hydroPHOBIC
Glutamic Acid is hydroPHILIC
What complications can sickle cells cause?
Odd shape, rigidity causes Increased blood viscosity
Stasis
Obstruction of arterioles and capillaries
Ischemia
Fragile= increased hemolysis
What are the steps to a vaso-occlusive crisis?
Vascular occlusion –> tissue hypoxia–> tissue necrosis–> pain
*Tissue necrosis can cause ulcers**
What are symptoms of vaso-occlusive crisis?
Symmetrical painful swelling of hands & feet
increased infection risk
Painful bones, joints, abdomen, back, & viscera
What are the CBC results for someone in SCT/SCA crisis?
Moderate to severe anemia
RBC indicies WNL
Leukocytosis
Thrombocytosis
PBS= Sickle cells
What test seems odd to be decreased in SCA but is possible due to the function of the RBCs?
Decreased ESR= Sickle cells can NOT form a rouleaux/stack due to shape
What special tests are used to Dx Sickle Cell Anemia/Trait?
Hb electrophoresis
What would the Hb Electrophoresis test results be for SCA & SCT?
SCA= 80-100% Hb S, no Hb A
SCT= 20-40% Hb S, 60-80% Hb A (still producing Beta Chains, just not as effectively)
What percentage needs to be hit for HB S to present as an anemia?
60% or more Hb S
What screening test is used for Hb S?
Sodium metabisulfite reduction test aka SickleDex Test
When do crises in SCA typically start?
1-2 years old
Thalassemia fits into what two categories of anemia?
Hypo/Micro
Hemolytic
What is Thalassemia?
genetic disorder that affects the RATE of adult Hb synthesis
Aka Cooley’s Anemia
Transfusions necessary to sustain life
B-Thalassemia Major
two bad copies of the gene, more severe.
Defective production rates of β-globin polypeptide
Leads to both decreased Hb production and excessive hemolysis
β-Thalassemia Major
Severe anemia
Jaundice- rate of destruction ⬆️
Splenomegaly
Hemolytic facies – maxillary hypertrophy, prominent forehead, can also have failure to thrive due to anemia
What is this?
β-Thalassemia Major
What CBC would β-Thalassemia Major have? PBS?
CBC= Severe Anemia w/⬆️RBC Count (yes, weird)
PBS= Target Cells
What special tests are used to Dx Thalassemia Major? What would you see?
Hb ELP (electrophoresis);
Significant reduction in Hb A w/ >50% of Hb F to compensate (up-regulation of Hb A2)
How do you treat thalassemia Major?
Transfusions to push Hb >9.3
Folate supplements
Treat infection quickly
Splenectomy
Iron MANAGEMENT- not supplements, avoid iron rich foods
β-Thalassemia Minor is frequently misdiagnosed as?
IDA
why does β-Thalassemia Minor usually go unnoticed?
asymptomatic or mild anemia
(certain stressors can change this)
What is this?
Mild anemia
Hb is typically >10
⬇️MCV/MCH/MCHC
RDW= WNL, can see a slight ⬆️ RDW at times
Slight ⬆️ RBC count
β-Thalassemia Minor
Anemia with an increased RBC count is unique to thalassemia conditions
what would a PBS of β-Thalassemia Minor look like?
Hypo/Micro RBCs
Possible= Target cell RBCs
What 3 special tests can you use to determine Thalassemia Minor?
Reticulocytosis count
Hb ELP
Calculate Mentzer index
How do you calc Mentzer index? What do the results mean?
MCV/RBC count
>13 IDA
<13 Thalassemia
How do you Tx Thalassemia Minor?
No Tx, live healthy, minimize stress
What does Thalassemia Minor look like on an iron panel?
WNL iron panel
its not an iron issue, its Hb Rate. Iron supplements can make them worse
Traumatic Hemolytic Anemia results from?
physical trauma
What are the lab findings for Traumatic Hemolytic Anemia?
anemia, ⬇️ MCV, ⬆️ RDW
PBS Schistocytes
The AKA for Traumatic Hemolytic Anemia is?
microangiopathic anemia
Transfusion of incompatible blood types results in?
Isoimmune hemolytic anemia
What are the 2 causes of hemolytic diseases in newborns?
ABO incompatibility
Rh Incompatibility
What are the lab findings for Isoimmune anemia?
CBC:
Anemia
Elevation of unconjugated bilirubin
Special tests:
Coomb’s test (direct anti-globin test aka DAT)
Will be + in our immune mediated hemolytic anemias
Acquired anemia induced by binding autoantibodies to RBC membrane is called?
Autoimmune hemolytic anemia (AIHA)
Two types of AIHA are?
Warm (IgG antibody)
Cold (IgM antibody)
Warm AIHA is which antibody and common with what?
IgG antibody
common with other autoimmune conditions
Cold AIHA is caused by and common in? What antibody?
Cold exposure, MC in elderly, acrocyanosis, Raynaud’s; IgM antibody
What are the risk factors for AIHA?
Autoimmune disorders
Prior blood transfusion
Malignancy
Medications
What test will be positive with Warm or Cold AIHA?
Direct Coombs Test (DAT- Direct Antiglobulin Test)
What kind of anemia will be seen with AIHA?
Normo/Normo
