Heme/Onc Flashcards
typical features of multiple myeloma (think CRAB)
hyperCalcemia (from osteoclast release by osteoclast activating factor IL-1, IL-6)
Renal insufficiency (Ig light chains in urine - Bence Jones protein)
Anemia, primary Amyloidosis (beta2-microglobulin)
Bone lytic lesions (punched-out) + Back pain
also, M-spike
increased suscept to infections
Rouleaux formation (RBCs stacked like poker chips)
eosinophils contain
histaminase
arylsulfatase
limits rxn following mast cell degran
basophils contain
heparin
histamine
leukotrienes (LTD4)
mast cells can bind what?
bind Fc portion of IgE onto membrane –> when IgE cross-links upon antigen binding –> degranulation
fibrin mesh formed by what bonds
covalent
proteins C + S inactivate what factors
Va
VIIIa
warfarin inhibits what enzyme
epoxide reductase
antithrombin inhibits activated forms of factors:
II VII IX X XI XII
vit K deficiency increases what lab values
PT and PTT!!
factor w/ shortest 1/2 life
VII
what are located inside endoth cells
tPA
thromboplastin (tissue factor)
vWF
PGI2
acanthocytes (spur cell) seen w/
liver disease
abetalipoproteinemia
IRREG spikes
basophilic stipping is what? seen w/?
aggregates of rRNA
Thalessemias
Anemia of chronic dz
Lead Poisoning
Alcoholism
“ACTors are based in LA”
bite cells seen w/
G6PD def
elliptocyte (aka pencil cell) seen w/
hereditary elliptocytosis
macro-ovalocytes seen w/
megaloblastic anemia
marrow failure
Ringed sideroblasts
**iron granules surround/encircle nucleus
sideroblastic anemia
lead poisoning
drugs, genetic conditions, myelodysplastic synd
schistocyte seen w/
DIC
TTP/HUS
traumatic hemolysis (metal heart valve prosthesis)
micro/macroangiopathic anemia
echinocyte (burr cell) seen w/
uremia renal failure pyruvate kinase deficiency microangiopathic hemolytic anemia mechanical trauma (prosthetic valves)
REG spikes
spherocytes seen w/
hereditary spherocytosis autoimmune hemolysis (warm agglut type)
teardrop cells seen w/
myelofibrosis
bone marrow infiltration
target cells seen w/
HbC disease
Asplenia
Liver disease
Thalessemia
Heinz bodies are what? seen in?
oxidation of Hb sulfhydryl groups –> denatured Hb precipitation + damage RBC membrane –> form bite cells (when it goes thru spleen)
seen in G6PD def
Howell-Jolly bodies are what? seen in?
basophilic nuclear remnants in RBCs
seen w/ asplenia or w/ mothball digestion (naphthalene)
disting b/w Howell-Jolly bodies vs. Heinz bodies?
only ONE HJ body per cell; MANY Heinz bodies per cell
cause of alpha thal
deletions in alpha-globin genes
4 alpha alleles on chrom 16
cause of beta thal
point mutations in splice sites + promoter seq
2 beta genes on chrom 11
primary Hb seen w/ Beta-thal minor
HbA2 (alpha 2, delta 2)
primary Hb seen w/ Beta-thal major
HbF
lead inhibits what enzymes
ALA-D (buildup of ALA)
ferrochelatase (buildup of protoporphyrin)
lead poisoning sympt
Lead lines on gingivae or metaphyses of long bones
Encephalopathy and Erythrocyte Basophilic stippling
Abd pain and sideroblastic Anemia
Drops - wrist/foot drop
sideroblastic anemia etiology + tx
X-linked defect in ALA-S gene
acquired: alcohol, lead, INH (causes B6 def)
tx = B6
what does hepcidin do in anemia of chronic dz
limits Fe transfer from macrophages to erythroid precursors by binding ferroportin on intestinal mucosal cells (inhib Fe transport) + suppresses EPO prod
folate vs B12 def (non-symptoms)
folate:
- green vegetables/fruit
- absorbed in JEJUNUM
- normal methylmalonic acid levels
B12:
- animal products! (strict vegans often affected)
- absorbed in ILEUM
- increased methylmalonic acid levels
tx for orotic aciduria
uridine monophosphate
nonmegaloblastic macrocytic anemia causes =
liver dz
alcoholism
reticulocytosis
drugs (5-FU, AZT, hydroxyurea)
signs of intravascular hemolysis
decreased haptoglobin increased LDH Hb in urine hemoglobinemia hemosiderinemia
signs of extravascular hemolysis
increased LDH
increased unconj bilirubin –> jaundice!
splenomegaly
increased risk of gallstones
causes of aplastic anemia
radiation
drugs (benzene, chloramphenicol, alkylating agents, antimetabolites)
virus (parvovirus B19, EBV, HCV, HIV) - esp in sickle cell pts
Fanconi’s anemia (DNA repair defect)
Idiopathic (primary stem cell defect - ex. telomerase def)
autoimmune
ass w/ pure red cell aplasia
thymomas
parvovirus B19
lymphocytic leukemias
PNH lab findings/tx?
CD55/59 negative on RBCs!!
tx = eculizumab
PNH triad?
hemolytic anemia
pancytopenia
venous thrombosis
“crew cut” on skull x-ray due to marrow hyperplasia seen in:
sickle cell anemia
beta-thal major
complic in sickle cell anemia
- aplastic crisis (parvo B19)
- **autosplenectomy (shrunken fibrotic spleen due to multiple infarcts) - increased suscept to encapsulated organisms; Howell-Jolly bodies seen
- splenic sequestration crisis - wedge-shaped infarcts + decreased H&H
- Salmonella osteomyelitis
- painful crises (dactylitis, acute chest synd, avascular necrosis usually of femoral head)
- renal papillary necrosis (due to low O2 in papilla) + microhematuria
what can cause both warm or cold type AIHA?
EBV, CLL
causes of warm agglutinin AIHA
SLE
CLL
alpha-methyldopa
infections/viruses
causes of cold agglutinin AIHA
CLL
Mycoplasma pneumo
infectious mononucleosis
microangiopathic anemia ass w/
DIC
TTP-HUS
SLE
malig HTN
macroangiopathic anemia ass w/
prosthetic valves
aortic stenosis
infections that destroy RBCs
malaria
Babesia
transferrin levels are a PRIMARY increase in what conditions
preg + OCP use
acute intermittent porphyria sympt + tx
5P's: Painful abd Port-wine colored urine Polyneuropathy (peripheral) Psych disturbances Precip by drugs
tx = glucose + heme (inhibs ALA-S)
acute intermittent porphyria enzyme affected + substrate accum =
enzyme = porphobilinogen deaminase (aka uroporphyrinogen-1-synthase)
accum =
- porphobilinogen
- ALA
- uroporphyrin (in urine)
porphyria cutanea tarda enzyme affected + substrate accum =
enzyme = uroporphyrinogen decarboxylase
accum = uroporphyrin (tea-colored urine!!!!!!)
porphyria cutanea tarda sympt + ass w/?
***blistering cutaneous photosensitivity! (from ROS damage)
hypertrichosis (excessive hair growth)
facial pigmentation
ass w/ HCV, alcoholism, increased LFTs
ALA-S needs what cofactor
B6
Bernard-Soulier synd
decreased GpIb –> decreased platelet-vWF adhesion
see: mild TCP w/ ENLARGED platelets
Glanzmann’s thrombasthenia
decreased GpIIb/IIIa –> defect in platelet-platelet aggreg
Lab = NO platelet clumping
ITP
anti-GpIIb/IIIa Ab (IgG)
increased megakaryocytes
usually wks after viral infection or immunization
TTP
deficiency of ADAMTS13 (vWF metalloprotease) –> large vWF multimers (the NON-active form) –> increased platelet aggreg –> thrombosis
labs: schistocytes!, increased LDH
sympt of TTP
pentad: Neuro sympt Fever TCP Hemolytic anemia Kidney damage
“Nasty Fever Torched His Kidneys”
blood transfusion risks =
infection transmission (low)
transfusion rxns
Fe overload
hypocalcemia (due to citrate = Ca + Mg chelator)
hyperkalemia (RBC lysis in old blood units)
cryoprecipitate contains
fibrinogen factor VIII factor XIII vWF fibronectin
Reed-Sternberg cells = + for what markers
CD30+
CD15+
both B-cell origin
marginal zone lymphoma ass w/?
chronic inflamm conditions like:
Hashimoto’s thyroiditis
Sjogren’s dz
H.pylori gastritis
Waldenstrom’s macroglobulinemia
IgM M-spike
NO lytic bone lesions
visual/neuro deficits + bleeding
Hairy cell leukemia unique features/tx
absent LAD!!
dry tap w/ bone marrow aspiration
TRAP +
tx = cladribine (adenosine deaminase inhibitor) or adenosine analog
common presentation of M3 AML
DIC
characteristic increase in what cell in CML
basophils
RF for CML =
ionizing radiation
benzene
Langerhans cell histiocytosis features?
lytic bone lesions + skin rash in child
do not efficiently stim T-lymphocytes via APC
cells express S-100 (neural crest origin) + CD1a
Birbeck granules!!
HIT caused by:
Ab-heparin-PF4 complex –> activate platelets –> thrombosis, TCP
cilostazol, dipyridamole MOA
PDE3 inhib –> increases cAMP in platelets –> inhib aggreg
also, arterial vasodilators
tx for CLL
fludrabine
prevent cardiotox induced by doxorubicin
dexrazoxane (iron chelating agent)
