CIS: RBC Disorders Flashcards
If on a deserted island and you have anemia, what is the one test that you would order?
The reticulocyte count
-represents immature blood cells
RBC’s live in blood how long?
120 days
What is a RBC 1-2 days before it is fully mature?
A reticulocyte
-we can test for reticulin
Aplastic anemia
The congenital kind is Fanconi anemia
- most cases are secondary to other causes
- EBV, HIV, radiation, chloramphenicol, quinacrine
What is chloramphenicol?
An antibiotic
- risk is 1:30,000 for aplastic anemia
- bone marrow replacement is a good treatment for it
What do people who sniff glue have increased risk for?
Aplastic anemia
What has aplastic anemia as a hallmark of the disease?
PNH
What will we see on a blood smear in chronic kidney disease
- spikes cells… Acanthocytes
- nor chromic normacytic anemia
Megaloblastic anemias
95% due to deficiency of either B12 or folic acid
-rest are due to drugs
What does the neuro symptoms with B12 patients look like?
- tertiary syphillis related tabes dorsalis
- so always check B12 levels
- the difference is that in tabes, there will be more urinary incontinence symptoms
What lab test do you do for folic acid deficiency?
Folate levels!
Tx of folate deficiency
Supplemental folate… 1mg/day
what is B12 deficiency due to ?
- impaired absorption
- pernicious anemia
- gastrectomy
- Zollinger-Ellison syndrome
- blind loop syndrome
- tapeworm infestation
- being a vegan….
What is pernicious anemia
-auto antibodies directs against PARIETAL CELLS, not Intrinsic factor itself
Iron deficiency anemia
- dietary deficiency: most infants have problems
- if bleeding from GI tract, it’s cancer until proven otherwise
What is Asthenia?
- pale
- ppl look like their just a bag of bones
What is the result of an altered expression of nRAMP2 gene?
- that encodes the DCT1 transport protein
- iron deficiency anemia
What is myoglobin
It’s in the muscles and gives them a store of Oxygen
-this is why iron deficient people have sore muscles
What do iron deficient people like to eat a lot?
- dirt (pica)
- ice
Is iron supplementation effective in anemia of chronic disease?
No
-if anemia is that severe, transfusions may be helpful to keep Hb in check
What is a myelopthisic anemia?
Anemia due to infiltration of the bone marrow
-like, by cancer or something
What are the ice cravings seen in iron deficiency anemia called?
Pagophagia
Clinical triad of diabetes
Polyuria, polydipsia, and something else
What the hallmark disorder for enzyme deficiency blood disorder
G6PD deficiency
- X-linked
- fava beans!!! Favism
What are Heinz bodies?
Collection of denatured hemoglobin
-spleen removes this and results in “bite cells”
March hemoglobinuria
- feet striking the ground repeatedly causes damage/lysis of RBCs in capillaries on plantar surface of feet
- common in marathon runners
- treat with well padded footwear and reassurance
- originally described in soldiers who developed hemoglobinuria after long marches
What was some of the chemical induced hemolytic anemias caused by?
Lead!
-slows production of RBC’s in marrow
Copper
What is the most common cause of hemolytic anemia in the world?
Malaria
- backwater fever
- their urine gets REALLY dark
Bartonellosis
- Bartonellosis bacilli forms
- does not infect, but adheres to RBC membrane
- hemolysis is the initial stage of the infection (Oroya fever)
- transmitted by sand flea
- treatment with Abx and supportive care
Babesiosis
- intraerythrocytic protozoa
- rodents
- “Maltese cross” appearance in RBCs with Giemsa-stained peripheral smears
What is a normal hemoglobin level in a woman
About 13 or so
-remember that if it’s like 6 or something, they will be VERY sick… Probably not walking into the clinic, but carried by a stretcher lol
If someone has hemolysis, what will the LDH level look like?
- it will be elevated
- it is normally around 200 or so
What will the reticulocyte count do when hemolysis is going on?
It will increase
- like up to 4%
- not 12 or anything like that
What do we look for in the indirect coomb’s test?
We look for antibodies that could potentially bind RBCs IN THE SERUM
-if it’s direct, we are looking on the red blood cell
What is characteristic of HUS?
- purpura
- acute kidney injury!
Immune mediated hemolytic anemia
- cold reacting antibodies… IgM
- simple meant may directly lose RBCs or opsonize RBCs for lysis in the spleen
- positive Indirect anti globulin test (coomb’s test)
Tx of warm antibody hemolysis
- most pts are not in imminent danger
- high dose steroids are the mainstay of therapy
WHAT can cause cold agglutinin disease?
Mycoplasma
-walking pneumonia
Acute intermittent porphyria AIP
- Scandinavian
- deficiency of porphobilinogen deaminase
- results in excretion of increased ALA and PBG in urine
- symptoms rare before puberty
- abdominal pain is most common symptom
