RBC disorders CIS Flashcards
Anemias- pathophysiologic categories
- by Retic count!!
- dec red cell prod
- inc red cell destruction or loss
Aplastic Anemia- characterized by?
-loss of hematopoietic cells, fatty replacement of marrow, and pancytopenia
Aplastic anemia- causes?
- may be congenital, but most secondary
- infection- viral hepatitis, EBV, parvovirus, HIV
- radiation
- drugs- Chloramphenicol, quinacrine!! (1 in 30,000)
- chemicals
- toluene
- glue
- immunologic- autoimune, PNH
Chronic kidney dz- causes anemia how?
- shortened RBC survival time
- dec EPO levels
- dec RBC prod indep of EPO
Chronic kidney dz- clinical manifestations
- normochromic normocytic anemia
- acanthocytes on PS (spikey cells)!!!!
- renal failure
megaloblastic anemia- causes?
- Vit B12 or folic acid def (95%)
- drugs or toxins (arsenic)
megaloblastic anemia- clinical aspects
- neuro sx- B12 def- degeneration of dorsal/lateral columns
- psychosis (severe B12 def)
refractory megaloblastic anemia- caused by?
myelodysplasia
Disturbance of Hemoglobin Synthesis
- iron def
- hemoglobinopathies
- porphyrias
Iron def anemia- caused by?
- most common cause of anemia in world!!
- dietary def- infants (bottle milk)
- pregnant women, children dont get enough in diet
- GI bleeding- most common cause!!!
Factitious anemia- due to?
auto phlebotomy
- Lasthenia de Ferjol syndrome
- psychiatric problems
iron def anemia- other causes?
- hemolysis/hemoglobinuria (PNH)
- post gastrectomy- insuff acid to maintain iron in Fe2+ state
- intestinal resection
- acquired- NRAMP2 gene- facilitates Fe2+ transport across brush border
iron def anemia- lab
- dec serum iron and ferritin
- inc TIBC
anemia of chronic dz- etiology
- dec RBC survival time
- dec erythropoiesis
- disturbed iron metabolism
anemia of chronic dz- lab
- dec serum iron and TIBC
- normal/inc serum ferritin
- normocytic normochromic anemia
anemia of chronic dz- tx
- malignancy
- benign- myelofibrosis, gaucher’s, histiocytosis, sarcoidosis
ice cravings
-pagophagia
enzyme def
- G6PD
- PK
- classic presentation- hereditary nonspherocytic hemolytic anemia!!
G6PD def
- food/drug induced
- FH (x-linked)
- Heinz bodies (denatured hemoglobin)
- bite cells
G6PD def- lab
- inc retic count
- hemolysis- dec haptoglobin, inc LDH and bilirubin, urine hemosiderin inc
G6PD def- tx
- avoid food/drugs that cause hemolysis
- splenectomy help some
March hemoglobinuria
(sports anemia, footstrike hemolysis)
- in marathon runners!!
- feet striking ground- lysis of RBCs in capillaries
Cardiac anemia
-severe aortic stenosis and prosthetic valves
chemical induced anemia
- lead
- copper
- oxygen- Astronauts!! (pure oxygen atmospheres)
- insect venoms- Bee/wasp, spiders
lead- anemia
- interferes with cation pump- shortened RBC survival time
- slows prod of RBCs in marrow
most common cause of hemolytic anemia in the world??
Malaria!!
-urine- “blackwater fever”- contains hemoglobin, very dark color
Bartonellosis
(Bartonella bacilliformis)
- adheres to RBC membrane
- transmitted by sand flea
- deadly!
Babesiosis
(Babesia microti)
- NE US!!
- parasite in rodents
- “Maltese Cross” in RBCs with Giemsa-stained peripheral smears!!!!
Immune mediated hemolytic anemia- caused by?
- warm reacting ab’s- IgG
- cold reacting ab’s- IgM- complement!
Immune mediated hemolytic anemia- clinical features
- positive direct antiglobulin test (DAT, Coombs)- Ig coats RBC surface
- indirect Coomb’s test- RBC-targeted Ig on serum
- normochromic normocytic anemia
- elevated retic count
- hemolysis evidence on lab tests
Tx of warm ab hemolysis
- not in imminent danger
- Steroids!! (high dose)- mainstay of tx!!
- immunosuppressives (cyclophosphamide, azathioprine)- considered in refractory cases
- splenectomy- in pts where chronic steroid use is required
cold ab hemolytic anemia- etiology
- lymphoprolif disorders!!
- cold agglutinin dz (Mycoplasma!!)
- tertiary syphilis!! (Donath-Landsteiner)
cold ab hemolytic anemia- tx
- avoid cold
- tx infections
Major porphyrias
- Acute intermittent porphyria
- porphyria cutanea tarda
Acute intermittent porphyria- caused by?
- def of PBG (porphobilinogen) deaminase
- inc incidence in psychiatric pts
- European descent
- excretion of inc amts of ALA and PBG in urine
Acute intermittent porphyria- clinical features
- sx usually after puberty
- abd pain- most common sx!!
- occasional attacks
- tachycardia, HTN
- neuro changes- peripheral neuropathy, anxiety, insomnia, depression, hallucinations, seizures
Acute intermittent porphyria- precipitating factors?
- hormones- pregnant women
- drugs- anticonvulsants, barbiturates, sulfonamides, alcohol
- low caloric intake
- infection
- surgery
Acute intermittent porphyria- tx
- hydration with carbohydrate solutions
- Hemin infusion (dec porphyrin prod via neg feedback)
- beta blockers- for tachycardia and HTN
- analgesics
Porphyria cutanea tarda- caused by? clinical?
def of uroporphyrinogen decarboxylase in liver
- cutaneous photosensitivity- major sx!
- no neuro effects
- cutaneous bulla formation on sun-exposed areas
- “pseudoscleroderma”
Polycythemia Vera- clinical presentation
- facial rubor!!
- hyperviscosity signs- HA, dizziness, blurred vision
- pruritus with hot shower or bath!!
Polycythemia Vera- lab
- inc RBC’s
- elevated LAP (leukocyte alkaline phosphatase) score
- inc WBC and/or plt counts in 50%
Polycythemia Vera- tx
- phlebotomy- lower RBC mass!
- hydroxyurea
