Hematology Flashcards
what is the final/main diagnostic test for most hematologic disorders?
bone marrow biopsy
What 3 nutritional cofactors are important in making heme and are often in iron supplements?
B6
glycine
succinate
what is normal TIBC? what would an increased or decreased value mean?
normal: 255-450 mcg/dL
increased: iron def anemia, PG, hormonal birth control
decreased: anemia of chronic disease, sideroblastic anemia, hemochromatosis
How is % iron saturation calculated
serom iron / TIBC
what are normal ferritin levels? when is ferritin often elevated or low?
normal: 20-300 (ideal 50-100)
elevates during inflammation/cancer
low during iron def/anemia
What pathologies could lead to an increased RBC count?
polycythemia vera
erythrocytosis
What pathologies could lead to an increased hematocrit (RBC mass)?
polycythemia vera
erythrocytosis
dehydration
shock
What pathologies could lead to an increased hemoglobin (blood concentration)?
polycythemia vera
severe burns
COPD
CHF
What pathologies could lead to an increased MCV?
macrocytic anemia
What pathologies could lead to an increased MCHC?
spherocytosis
What pathologies could lead to an increased MCH?
macrocytic anemia
What pathologies could lead to a decreased RBC count?
anemia
blood loss
lymphoma
myeloproliferative disorder
leukemia
What pathologies could lead to a decreased hematocrit?
anemia
leukemia
acute blood loss
What pathologies could lead to a decreased hemoglobin?
anemia
hemolytic rxns
hemorrhage
system disease
What pathologies could lead to a decreased MCV?
microcytic anemia
What pathologies could lead to a decreased MCHC?
iron def
macrocytic anemia
thalassemia
What pathologies could lead to a decreased MCH?
microcytic anemia
factors that can interfere with testing RBC count
dehydration
age
altitude
pregnancy
factors that can interfere with testing hematocrit
dehydration
age
altitude
pregnancy
factors that can interfere with testing hemoglobin
altitude
excess fluid
pregnancy
drugs
factors that can interfere with testing MCV
can be normal in normocytic anemia
factors that can interfere with testing MCHC
presence of cold agglutins, lipemia, high amounts of heparin
factors that can interfere with testing MCH
hyperlipidemia
normal hematocrit
F: 36-48
M: 42-52
normal hemoglobin
F: 12-16
M: 14-17
normal MCV
82-98
normal MCHC
31-37
normal MCH
26-34
normal RBC count
F: 3.6-5 x 10^6
M: 4.2-5.4 x 10^6
What pathologies could lead to an increased RDW?
iron def
vitamin B12/folate def
thalassemia
What pathologies could lead to an increased platelet count?
malignancy
myelogenous leukemia
polycythemia vera
What pathologies could lead to an increased WBC count?
infxn
leukemia
malignant neoplasms
What pathologies could lead to an increased neutrophil count?
bacterial infxns
inflammation
leukemia
What pathologies could lead to increased lymphocytes?
leukemia
lymphoma
mononucleosis
viral dz
What pathologies could lead to increased monocytes?
TB
subacute bacterial endocarditis
leprosy
lipid storage dz
some leukemias
What pathologies could lead to increased eosinophils?
allergies
parasite infxn
skin dz
infxn
What pathologies could lead to increased basophils?
granulocytic and basophilic leukemia
myeloid metaplasia
hodgkins lymphoma
What pathologies could lead to decreased RDW?
posthemorrhagic anemia
What pathologies could lead to decreased platelets?
idiopathic thrombocytopenia
purpura
exposure to DDT
chemo
What pathologies could lead to decreased WBC?
viral infxn
bone marrow depression/disorders
What pathologies could lead to decreased neutrophils?
drugs
chemicals/radiation
blood dz
acute relentless bacterial ifxn with poor prognosis
What pathologies could lead to decreased lymphocytes?
chemo
steroid administration
tumor
malignancy
What pathologies could lead to decreased monocytes?
prednisone use
hairy cell leukemia
RA
HIV
What pathologies could lead to decreased eosinophils?
cushings syndrome
medications
What pathologies could lead to decreased basophils?
acute infxn
stress
steroid therapy
factors that can interfere with testing RDW
result isnt useful if anemia is not present
factors that can interfere with testing platelet count
count inc after exercise, in winter, and at high altitudes
factors that can interfere with testing WBC count
age, time of day
factors that can interfere with testing neutrophils
steroid administration
extreme heat or cold
age
factors that can interfere with testing eosinophils
stress
time of day
normal values RDW
11.5-14-5%
normal values platelets
140-400 x 10 ^3
normal values WBC
5-10^3
normal ratio of WBC
“never let me eat blood”
N: 50-60
L: 20-40
M: 2-6
E: 1-4
B: 0.5-1
what is meant by “left shift”
over 6% immature band neutrophils
indicates bacterial infxn
what is meant by “right shift”
hypersegmentation of neutrophils
indicates B12/folate deficiency
What hemotologic disorders would you be most suspicious of if a patient was reporting pain?
macrocytic anemias
leukemias
sickle cell anemia
multiple myeloma
What hemotologic disorders would you be most suspicious of if a patient was reporting fatigue
microcytic and macrocytic anemias
leukemias
mono
lymphomas
What hemotologic disorders would you be most suspicious of if a patient was reporting purpura
senile purpura
ITP/TTP
true clotting disorders: VWF, hemoA&B
What hemotologic disorders would you be most suspicious of if a patient was reporting GI complaints
pernicious anemia
What hemotologic disorders would you be most suspicious of if a patient was reporting lymphadenopathy
lymphomas
mono
What hemotologic disorders would you be most suspicious of if a patient was exhibiting hepatomegaly or splenomegaly
both: leukemias
spleen only: mono
basic labs for heme
CBC w differential and PLT
reticulocyte count
ferritin, TIBC
aside from initial work up, what additional testing would you consider for suspected macrocytosis?
MMA
neutrophil segmentation
B12 and folate levels
aside from initial work up, what additional testing would you consider for suspected microcytosis?
iron studies
RBC morphology
reticulocyte counts
erythropoetin levels
aside from initial work up, what additional testing would you consider for lymphadenopathy?
WBC morphology
EBV and CMV viral studies
bone marrow studies
aside from initial work up, what additional testing would you consider for hemolysis
indirect and direct bilirubin
RBC morphology and membrane studies
reticulocyte count
aside from initial work up, what additional testing/considerations would you have for pain
consider B12 anemias
urine electrophoresis (bence jones protein)
R/O hemolytic anemias
aside from initial work up, what additional testing/considerations would you have for neuro sx
macrocytic anemia work up
anemia is defined how?
low HCT and Hb
name the microcytic anemias (low MCV)
iron def
thalassemia
sideroblastic
lead poisoning
name the normocytic anemias (normal MCV)
acute blood loss
hemolysis
anemia of chronic disease
anemia of renal failure
myelodysplastic syndromes
name the macrocytic anemias (high MCV)
folate def
b12 def
drug toxicity (e.g. zidovudine)
alcoholism/chronic liver dz
typical values of the following for iron def anemia:
- smear
- SI
- TIBC
- % sat
- ferritin
- smear: microcytic/hypochromic
- SI: <30
- TIBC: >360
- % sat: <10
- ferritin: <15
typical values of the following for thalassemia:
- smear
- SI
- TIBC
- % sat
- ferritin
- smear: microcytic/hypochromic with targeting
- SI: normal to high
- TIBC: normal
- % sat: 30-80
- ferritin: 50-300
typical values of the following for sideroblastic anemia:
- smear
- SI
- TIBC
- % sat
- ferritin
- smear: variable
- SI: normal to high
- TIBC: normal
- % sat: 30-80
- ferritin: 50-300
typical values of the following for inflammation:
- smear
- SI
- TIBC
- % sat
- ferritin
- smear: normal microcytic/hypochromic
- SI: <50
- TIBC: <300
- % sat: 10-20
- ferritin: 30-200
microcytic hypochromic anemias
iron def
thalassemia
chronic infxn
severe protein def
what pathologies would you consider in a normocytic, normochromic anemia with high reticulocytes?
lost blood cells
- acute blood loss
- hemolytic anemia
what pathologies would you consider in a normocytic, normochromic anemia with low reticulocytes?
underproduction
- red cell aplasia
- drugs, leukemia, aplasitic anemia
- chronic dz, liver or kidney dz
macrocytic anemia etiology and diagnostic testing
vitamin B12 def; may be folic acid responsive
dx:
- elevated MCV, low reticulocyte count
- hyper segmented (>5) neutrophils
- low b12 levels
- low MMA (serum)
what is the most common hemolytic anemia? what would you see on a peripheral smear of someone with this diagnosis?
G6P deficiency
bite cells or heinz bodies on peripheral smear
would a G6PD patient appear anemic?
not unless exposed to oxidant drugs; which is why its important to check RBC G6PD prior to high dose vit C IVs
why does G6PD offer a selective advantage against malaria?
malaria infests the RBC
etiology sickle cell disease
replacement of glutamic acid by valine at position 6 of the B chain leading to hemoglobin S
signs/sx sickle cell
occlusion of precapillary arterioles
anemia and splenomegaly
attacks of pain in chest, abdomen, skeleton
multiple infarctions in bone marrow
hemochromatosis etiology and clinical picture
AR (chromosome 6, HLA-A3) disorder that results from excessive iron absorption from food; cant get rid of iron and it oxidizes and deposits in skin/other tissues
manifests typically at 40-60, more commonly in men
liver failure (cirrhosis)
pancreatic failure (“bronze diabetes”)
epoetin alfa indications / use
anemia related to:
- chronic renal failure
- zidovudine therapy in HIV pts
- chemotherapy in pts with metastatic nonmyeloid malignancies
reduction of allogenic blood transfusions in surgery pts
unlabeled uses: anemia for chronically ill pts, CHF, chronic dz, postpartum anemia, sickle cell, thalassemia, jehovahs witnesses
what dx of are dx of exlusion when you have ruled out all other causes for purpura?
purpura simplex
senile purpura
henoch schonlein purpura etiology and clinical picture
inflammatory disorder of unknown cause with IgA complexes in capillary beds/joints
acute respiratory infxn usually precedes the purpura
purpuric rash on LEs
abdominal pain or renal involvement
arthritis
hematuria
ITP etiology and clinical picture
Ab form against platelets; frequently preceded by URI/viral infxn
presents as petechiae and other bleeding such as CNS bleeding or bleeding gums
TTP etiology and clinical picture
fatal; usually die at 30-40 with tx
due to inhibitor of vWF-cleaving protease and unchecked platlet aggregation
dx criteria:
microangiopathic hemolytic anemia (shistocytes, helmet cells on smear)
elevated LDH
mental status changes or fluctuating focal neuro deficits
hemolytic uremic syndrome etiology and clinical picture
hemolytic anemia from toxin > hemolytic cells plug up kidney > kidney failure
etiology:
bacterial: diarrheal illness from ecoli, shigella, staph
drugs: chemo, tacrolimus, ticlopidine, oral contraceptives
VWD etiology and clinical picture
hereditary abnormal synthesis of vWF > dec platelet adhesion and dec serum levels of factor VIII:C
hx heavy menses
epistaxis
easy bruising
GI bleeding
other testing normal; do clotting studies/vW profile
disseminated intravascular coagulation (DIC) etiology and clinical picture
Use up clotting factors > bleed out
- complication of obstetrics (abruptio placentae, saline aborrtion, retained products of conception, amniotic fluid embolism, eclampsia)
- infxn (esp gram neg with endotoxin release)
- malignancy (esp adenocarcinoma of pancreas and prostate, acute leukemia)
hemophilia A etiology, dx, tx
X linked recessive def of factor VIII
dx by factor VIII assay; PTT normal or elevated, PT and thrombin clot time normal.
tx:
factor VIII supplementation
hemophilia B (christmas disease) etiology, dx, tx
X-linked recessive def of factor IX
dx by factor IX assay, tx def
what condition most commonly causes relative/reactive polycythemia?
emphysema/COPD (rxn to inc erythropoietin)
NOT A CANCER
polycythemia vera pathophys/sx
Malignant stem cell disorder with increase in red cell mass (similar to a leukemia of RBC) and inc RBC/WBC/platelets
fatigue, weakness, dizziness, HA, visual problems
itching after warm bath
easy bruising or bleeding with little or no injury
multiple myeloma etiology and sx
neoplastic proliferation of a single clone of plasma cell producing monoclonal IgG or IgA
> 50, progressive onset
bone and back pain, unexplained fractures
bleeding problems
aggravation of arrythmias
multiple myeloma diagnostic
bone marrow biopsy
bone x ray (shows fractures, “punched out” bone lesions)
hypercalcemia
bence jones proteniuria (IgG/IgA inc protein secretion)
waldenstroms macroglobulinemia presentation and etiology
malignant disease of b lymphocytes with overproduction of IgM > hyper viscous blood and peripheral vascular compromise
similar multiple myeloma presentation but less common
what is the most common leukemia in children?
acute lymphocytic leukemia (ALL)
ALL etiology, sx, prognosis
fever, bone pain, hepatosplenomegaly
associated with down syndrome, radiation, viral infxns
90% remission with tx, 3-6 mo surivial without tx
what is the most common leukemia in adults 15-39?
acute myeloblastic leukemia (acute nonlymphocytic leukemia)
acute myleoblastic/nonlymphocytic leukemia presentation and prognosis
splenomegaly
auer rods in cytoplasm
may present with bleeding disorders or high WBC
cure rate 10-15%, 1 year survivial with chemo
what age is a typical pt with chronic myleogenous leukemia?
45
chronic myleogenous leukemia etiology, clinical presentation, and prognosis
well differentiated granulocytic leukemia (may include any cell line); slow for 3 years then “blast crisis”
90% of pts have the philadelphia (Ph) chromosome
hepatosplenomegaly, fatigue, generalized LA, weakness, anorexia/wt loss
4-6 year survival
chronic lymphocytic leukemia typically affects individuals around what age?
over 55
chronic lymphocytic leukemia sx
none
enlarged lymph nodes, liver, spleen
fatigue
abn bruising (late in dz)
night sweats
loss of appetite
wt loss
VERY inc WBC (50-250k) - often found incidentally
hodgkins lymphoma etiology and sx
Malignant proliferation of germinal center B cells
20 or 60 yo, curable, familial, prognosis depends on stage
single asx swollen node (unexplained lymphadenopathy)** > spreads.
Intermittent spiking fever, night sweats, wt loss
lymphocytopenia
reed sternberg cell with hodgkins disease
what is the most common lymphoma?
non-hodgkins lymphoma
non hodgkins lymphoma etiology and presentation
malignant growth of B or T cells
similar presentation to hodgkins but more deadly
associated with burkitts and immunoblastic lymphomas
burkitts lymphoma etiology and presentation
B lymphocyte tumor
lymphadenopathy in the maxilla or mandible
associated with EBV in US, malaria in africa
may predispose pt to NHL
Etiology, presentation, and prognosis acute lymphoblastic leukemia (ALL)
“ALL my children”, age 3-5
Associated w Down syndrome, radiation, viral infxns
Fever, bone pain, hepatosplenomegly
Good prognosis
Etiology, presentation, and prognosis Acute myeloid leukemia (AML)/ acute non lymphocytic leukemia
“All My Life”, age 15-39
Auer rods, Splenomegaly, bleeding disorders, inc WBC
Poor prognosis
Etiology, presentation, and prognosis Chronic myeloid leukemia (CML)
“I always have a BLAST eating CaraMeL in Philadelphia”
45 years old
Slow for 3 years > BLAST crisis (85% die); hepatosplenomegaly, fatigue, wt loss, weakness
Philadelphia (Ph) chromosome
4-6 year survival
Etiology, presentation, and prognosis Chronic lymphocytic leukemia (CLL)
“Cranky Late Lifers”; >60
Monoclonal disorder with progressive accumulation of functionally incompetent B cells
Can be asx, enlarged nodes/liver/spleen, systemic sx
May be found incidentally on CBC with inc WBC
Death from cytopenia secondary to bone marrow replacement from infections
Tx multiple myeloma
Non curative
Chemo, stem cell transplant <65
Bromelain
Kidney protection
Etiology, presentation, and dx/tx of babesiosis
Babesia parasites carried by ixodes ticks > destruction of RBCs
Asx or flu/malaria like sx
Dx via blood smear (Maltese cross, ring form, hemolysis)
Etiology, presentation, and dx/tx of Malaria
F anopheles mosquito transmits plasmodia to humans > infects RBC + liver > divides in RBC > RBC lyses > cont to infect next RBC
High fever + shaking chills, hepatosplenomegaly, abdominal pain, diarrhea, myalgia, HA, cough
Rapid antigen, blood microscopy (visible parasites), thrombocytopenia without leukocytosis
Tx: hydroxychloroquine
Forms of malaria
P Vivax/p ovale - chills, fever, q48h
P malariae - chills, fever, Q 72h
P falciparum - daily spikes with no pattern; most common/lethal - seizures, coma, renal failure, RDS
Etiology, presentation, and dx/tx of Septicemia
Bac endotoxin damages endothelial cells > release NO/PG2 > mast cells > TNF/IL1 from mo > vascular leakage >
Peripheral vasodilation w dec systemic vascular resistance > heart compensates inc HR/CO
Usu due to gram neg pathogens (e coli)
Sx: warm skin, bounding pulse, RDS, DIC, fever/chills, SOB, confusion
Work up: CBC, electrolytes, kidney/liver function, urine culture, wound culture
Tx: O2, IV fluids, IV abx ,ER
What is the difference between lymphadenitis and lymphangitis?
Phad = node
Phang = channel
Etiology, presentation, and dx/tx of Lymphadenitis
Inflammation lymph node
Painful, tender lymph nodes; fluctuating and warm, soft, firm, rubbery
Gram stain of aspirated tissue to r/o bacterial, monospot, or EBV
Tx: calendula, phytolacca, ceanothus americanus, galium aparine
Etiology, presentation, and dx/tx of Lymphangitis
Inflammation of lymph channel usu d/t cellulitis with strep pyogenes
Deep red skin, warmth, raised around area, high fever, pain
Leukocytosis, blood culture
Tx: IV abx if systemic, analgesics, hot compress, elevate
Etiology, presentation, and dx/tx of Lymphedema
Abnormal collection of protein rich fluid in interstitium restyling from obstruction of lymph drainage
Can be primary or secondary
Tx: PT, compression, hygiene/skin care
Primary and secondary causes of lymphedema
Primary - congenital hypoplasia/aplasia of peripheral lymphatics, valvular incompetence
Secondary:
Infxn (filariasis mosquito), Wicheria bancrofti (#1 cause worldwide; permanent lymphedema)
Malignant infiltration, obesity
Radiation/surgery (axillary, groin) - number 1 cause in NA
Etiology, presentation, RF a-thalassemia
Microcytic, hypochromic
Dec in a-globulin chain synthesis due to gene deletion
defective
- 1: clinically silent, normal MCV/Hb
- 2: dec MCV, normal Hb
- 3: dec MCV and Hb, splenomegaly
- 4: no chance of survival
Southeast Asian/african heritage
Work up, dx, and tx of a-thalassemia
Basophilic stippling, normal RDW, ferritin, reticulocytes
Microcytic, hypochromic anemia
Dx: DNA analysis with gene probes
Tx for 1-2: none, transfusion if severely anemic + sx
Etiology, presentation, RF For b-thalassemia
Microcytic hypochromic
Dec in beta-globulin chain synthesis due to gene deletion
Minor: heterozygous
Major: homozygous; dx 4-6 mo with severe anemia, jaundice, wasting, slow growth, delayed onset of secondary sex features
RF: Mediterranean
Etiology, presentation, RF Of B12 def anemia
Megaloblastic, microcytic
Causes: malnutrition, malabsorption, pernicious anemia, fish tapeworm, resection of ileum, inc utilization (pregnancy/lactation)
Sx: smooth sore tongue with atrophy of papillae, peripheral neuropathy (BL, reversible), confusion, CN optic atrophy
Inc MCV, dec reticulocytes, hypersegmented neutrophils (right shift)
Who should you never give a B12/folate supplement to?
Cancer pts
How is alcoholism / hypothyroid induced anemia different from the other macrocytic anemias?
NON-megaloblastic
Types of hereditary hemolytic anemia
Abnormal…
Membrane - spherocytosis
Enzyme - pyruvate kinase/G6PD def
Hemoglobin - thalassemias
Types of acquired hemolytic anemias
AI (warm IgG/cold IgM)
Drug induced
AI (transfusion rxn, Rh hemolytic disease of newborn)
Sx hemolytic anemia
Jaundice, hepatosplenomeglay, dark urine, cholelithiasis, iron overload w extra vascular hemolysis, iron def with intravascular hemolysis
Inc reticulocytes, unconjugated bilirubin
Normocytic anemia
Intravascular vs extravascular hemolytic anemia work up
IV: shistocytes, free Hg in serum
EV: direct Coombs/indirect Coombs
Tx hemolytic anemia
Disc drugs, splenectomy, IV immunoglobulin
Etiology, presentation, RF Aplastic anemia
Destruction of hematopoeitic cells > pancytopenia + hypocellular bone marrow
Idiopathic (T cell), meds (chemo, chloramphenicol), chemicals (DDT), infxn (EBV, CMV, parvo19, hep C, HIV), radiation, SLE (rare)
Pallor, patechaie,easy bleeding
Dec RBC, WBC, platelets, dec reticulocytes
Marrow cells replaced w fat
Tx aplastic anemia
Broadspectrum abx, transfusions, cyclosporine, BMT, GFs
causes of secondary polycythemia
Reaction to inc EPO
Renal artery hypoxia
Emphysema
Tumors
Tetralogy of fallot
Etiology, presentation, RF Vit K def
Fat malabsorption (celiac), biliary obstruction, prolonged abx tx, oral anticoagulants, poor diet, alcoholics
Sx: GI bleed, bleeding into subcutaneous tissue, bleeding at time of circulation, intracranial hemorrhage
Enzyme def in subtypes of porphyria
EPP: ferrochelatase
AIP: porphobilinogen deaminase
PCT: uroporphyrinogen decarboxylase
Age of onset in subtypes of porphyria
EPP: childhood or early adulthood
AIP: adolescent or adulthood
PCT: adulthood
Sx porphyrias
Photosensitivity, burning skin, skin/abdominal pain, neuro sx, hyperpigmentation
Trigger factors in subtypes of porphyria
EPP: sun
AIP: meds, alcohol, fasting
PCT: alcohol, estrogen, hep C, HIV
Skin involvement in subtypes of porphyria
EPP: non-blistering
PCT: blistering
Dx in subtypes of porphyria
EPP: inc protoporphyrin in blood/stool
AIP: inc PBG and ALA in urine
PCT: inc uroporphyrins in urine/plasma
Tx and prognosis for types of porphyria
EPP: sun protection, beta carotene, afamelanotide (can be managed)
AIP: IV glucose, hemin (can be fatal without tx)
PCT: phlebotomy, low dose antimalarials (good w tx)
