Heme/Onco Flashcards
CD 34+
hematopoietic stem cell
membrane contains Cl-/HCO3- antiporter
RBC
don;t produce net ATP during glycolysis
RBC
2,3, BPG in RBC
produced from 1,3 BPG by bisphosphoglycerate mutase using ATP
platelet contents
dense granules (ADP, Ca2+), alpha granules (vWF, fibrinogen)
where is 1/3 of platelets stored
spleen
blood cell differentiation fro highest to lowest
neutrophils, lymphocytes, monocytes, eosinophils, basophils
hypersegmented polys
B12/folate deficiency
high cortisol state and neutrophils
disrupts ability of neutrophils to adhere to wall of damaged tissue releasing them into circulation
frosted glass cytoplasm
monocytes
what activates macrophages
gamma interferon
surfacemarker for macrophages
CD 14
eosinophils produce
histaminase and arylsulfatase
causes of eosinophilia
neoplastic, asthma, allergic, collagen vascular disease, parasites
mediates allergic reaction
basophil
contain heparin, histamine, LTD4
basophil
basophilia
usually seen with CML
can bind Fc portion of IgE
mast cell
involved in type 1 hypersensitivity
mast cell
chromolyn sodium
prevents mast cell degranulation
increased production of gastric acid by parietal cells
systemic mastocytosis
most sensitive cells to radiation
lymphocytes
produces lymphocytosis promoting factor and blocks lymphocytes from leaving blood
Bordetella pertussis
express CD8, recognize MHC I
cytotoxic T cells
express CD28
regulatory T cells
universal plasma donor
AB
Rho immune globulin
given to mother at first delivery
clot instability, delayed, recurrent bleeding after trauma
factor XII deficiency
decreased ESR
polycythemia, sickle cell anemia, CHF, microcytosis, hypofibrinogenemia
acanthocyte (spur cell)
liver disease, abetalipoproteinemia
basophilic stippling
TAL: thalassemia, anemia of chronic disease, lead poisoning
bite cell
G6PD deficiency
elliptocyte
hereditary elliptocytosis
macro-ovalocyte
megaloblastic anemia (also hypersegmented PMNs), marrow failure
ringed sideroblasts
sideroblastic anemia (excess iron in mitochondria)
schistocyte
DIC, TTP/HUS, traumatic hemolysis
spherocyte
hereditary spherocytosis, autoimmune hemolysis
teardrop cell
bone marrow infiltration (myelofibrosis)
target cell
HALT: HbC disease, asplenia, liver disease, thalassemia
Heinz bodies
oxidation of iron, G6PD, similar in alpha-thalassemia
Howell-Jolly bodies
basophilic nuclear remnant normally removed by splenic macrophages; hyposplenia or asplenia, mothball ingestion (naphthalene)
Plummer-Vinson syndrome
iron deficiency anemia, esophageal webs, atrophic glossitis
koilonychia, pica
iron deficiency anemia
alpha thalassemia cis deletion
Asians, worse than trans
alpha thalassemia trans deletion
Africans
4 gene deletion alpha thalassemia
Hb Barts, hydrops fetalis
3 gene deletion alpha thalassemia
HbH
which chromosome is the mutations on in alpha thalassemia
16
which chromosome is the mutation on in beta thalassemia
11
point mutations in splice sites and promoter sequences
beta thalassemia
prevalent in mediterranean population
beta thalassemia
asymptomatic, increased HbA2 on electrophoresis, target cells
beta thalassemia minor
severe anemia, risk for aplastic crisis with parvovirus B19, marrow expansion
beta thalassemia major
increased HbF
beta thalassemia major
inhibits ferrochelatase and ALA dehydratase
lead poisoning
house with chipped paint, basophilic stippling
lead poisoning
lead poisoning
LEADS” lead lines on gingiva, encephalopathy and erythrocyte basophilic stippling, abdominal colic, sideroblastic anemia, drops (wrist, foot), dimercaprol and EDTA for treatment, succimer for chelation in kids
sideroblastic anemia causes
X-linked defect in gamma-ALA synthase, alcohol, lead, isoniazid
sideroblastic anemia treatment
B6 (cofactor for gamma-ALA synthase)
anemic of chronic disease treatment
exogenous erythropoetin
can cause folate deficiency
methotrexate, trimethoprim, phenytoin
macrocytic anemia, hypersegmented neutrophils, glossitis, increased homocysteine, normal methylmalonate
folate deficiency anemia
macrocytic anemia, hypersegmented neutrophils, glossitis, increased homocysteine, increased methylmalonic acid
B12 deficiency anemia (converts methylmalonic acid to succinyl CoA
normal Schilling test
low dietary intake of B12
cause of B12 deficiency
Crohns, pernicious anemia, Diphyllobothrium latum, PPI
can’t cleave off R-binder from B12
pancreatic insufficiency
megaloblastic anemia in a child that can’t be cured with folate or B12
orotic aciduria
hypersegmented neutrophils, glossitis, orotic acid in urine
orotic aciduria
orotic aciduria treatment
uridine monophosphate
decreased haptoglobin, increased LDH, Hn in urine
intravascular hemolysis
increased LDH, increased UCB, jaundice
extravascular hemolysis
correct for falsely elevated reticulocytes
multiply by Hct/45
master regulator of iron homeostasis
hepcidin (produced in the liver); inhibits iron transport across gut mucosa
anemia without splenomegaly
aplastic anemia
pancytopenia, dry bone marrow tap, pallor, purpura, mucasal bleeding
aplastic anemia
aplastic anemia causes
radiation, benzene, chroramphenicol, alkylating agents, antimetabolites, parvovirus B19, EBV, HIV, HCV, fanconi’s anemia (DNA repair defect)
thymoma
associated with pure red cell aplasia
complication of any hemolytic anemia
pigmented gallstones, predisposes to folic acid deficiency
autosomal dominant defect in spectrin, ankryrin, increased MCHC, premature removal of RBCs by spleen
hereditary spherocytosis
splenomegaly, aplastic crisis (B19), positive osmotic fragility test
hereditary spherocytosis
hereditary spherocytosis treatment
splenectomy
X-linked recessive decrease in glutathione
G6PD
hemolytic anemia following oxidant stress
G6PD
back pain, hemoglobinuria, Heinz bodies, bite cells, dercreased production of 6-phosphogluconate
G6PD
decreased ATP, hemolytic anemia in newborn
puryvate kinase deficiency
HbC
glutamic acid to lysine mutation at residue 6 in beta globin
complement-mediated RBC lysis, absent GPI
paroxysmal nocturnal hemoglobinuria
hemolytic anemia, pancytopenia, venous thrombosis
paroxysmal nocturnal hemoglobinuria
CD55/59 negative on flow cytometry
paroxysmal nocturnal hemoglobinuria
treatment for paroxysmal nocturnal hemoglobinuria
eculizumab
paroxysmal nucturnal hemoglobinuria screening
sucrose test
substitution of glutamic acid with valine at position 6 of beta chain
sickle cells anemia
low O2 in sickle cell
precipitates sickling–> deoxygenated HbS polymerizes–>promotes hydrophobic interaction among Hb molecules
crew cut skull on x-ray, aplastic crisis, autosplenectomy, increased risk of infection with encapsulated organisms, salmonella osteomyelitis, renal papillary necrosis
sickle cell anemia
sickle cell anemia treatment
hydroxyurea (increases HbF)
gardos channel blockers
hinder efflux of K+ and water from cells, preventing dehydration of RBCs and decrease polymerization of HbS
metabisulfite screen
causes cells with any amount of HbS to sickle, predisposed to develop folic acid deficiency
HbC
normocytic anemia with extravascular hemolysis, mutation in beta chain
IgG
extravascular hemolysis, spherocytes, warm agglutinin
IgM
intravascular hemolysis, cold agglutinin
warm agglutinin
SLE, CLL, alpha-methyldopa
cold agglutinin
acute anemia triggered by cold; CML, Mycoplasma pneuminia, mono
direct Coombs test
anti-Ig Ab added to pts serum
indirect Coombs test
normal RBCs added to pts serum
infectious mono
EBV infection, lymphocytic leukocytosis, reactive CD8+, generalized LAD (paracortex), splenomegaly (PALS), risk for splenic rupture
monospot test
detects IgM heterophile Ab; negative–>CMV cause
definitive diagnosis for mono
testing EBV viral capsid antigen
rash if exposed to penicillin
mono
often fatal leukemia, pigmentary skin changes, malformation of heart, kidney, and limbs
Fanconi anemia (mutation in gene that functions in BRCA)
BRCA
repairs damage to DNA caused by cross-linking agents
accumulation of protoporphyrin, gamma-ALA in blood
lead poisoning
defective porphobilibogen deaminase
acute intermittent porphyria
accumulation of porphobilinogen, gamma-ALA, uroporphyrin in urine
acute intermittent porphyria
acute intermittent porphyria treatment
glucose and heme (inhibit ALA synthase)
painful abdomen, port-wine colored urine, polyneuropathy, psychological disturbances, precipitated by drugs
acute intermittent porphyria
defective uroporphyrinogen decarboxylase
porphyria cutanea tarda
uroporphyrin in urine (tea-colored urine)
porphyria cutanea tarda
blistering cutaneous photosensitivity
porphyria cutane tarda
normal bleeding time
2-7 min
normal PT time
11-15 s
normal PTT time
25-40 s