Heme Flashcards
preferred anticoagulant for immediate anticoagulation
heparin
long-term anticoagulation
warfarin
what allows RBC to change shape in vessels
spectrin
lead poisoning
basophilic stippling
G6PD deficiency
Heinz bodies and bite cells
DIC
schistocytes
abetalipoproteinemia
acanthocytes (spur cells)
asplenia
howell-jolly bodies and target cells
liver disease
acanthocytes and target cells
bone marrow infiltration (myelofibrosis)
teardrop cell
Target cells
THAL
Thallasemia, HbC disease, Asplenia, Liver disease
absence of HGPRTase
Lesch-Nyhan
deficiency of aldolase B
fructose intolerance
deficiency of cystathionine synthase
homocystineuria
galactose-1-phophate uridyl transferase deficiency -> intellectual disability, HSM, cataracts
galactosemia
deficiency of tyrosinase
albinism
location of fetal erythropoiesis
Young livers synthesize blood yolk sac liver spleen bone marrow
different causes of polycythemia
increased EPO (tumors)
chronic hypoxia
polycythemia vera
trisomy 21
lead poisoning
hematologic: microcytic anemia, basophilic stippling, ringed sideroblasts
non-hematologic: encephalopathy, memory loss, HA, foot/wrist drop, lead lines, abdominal colick, renal failure
lead poisoning affects which enzymes
ferrochelatase and ALA dehydratase
leads to accumulation of protoporphyrin and d-ALA
acute intermittent porphyria
inhibits porphobilinogen deaminase (uroporphyrinogen-1-synthase)
accumulates porphobilinogen, d-ALA, and uroporphyrin
5 P’s:
painful abdomen, port-wine urine, polyneuropathy, psychological disturbances, precipitated by drugs
treatment of acute intermittent porphyria
glucose and heme (inhibits ALA synthase)
porphyria cutanea tarda
uroporphyrinogen decarboxylase
accumulates uroporphyrin
blistering cutaneous photosensitivity, hypertrichosis, facial hyperpigmentation, Hep C and alcoholism
RLS of Heme synthesis
d-ALA synthase (d-aminolevulinic acid)
diagnose beta-thalassemia minor
HbA2 on electrophoresis
lab findings of iron def:
decreased iron and ferritin, increased TIBC
what should be ruled out in 50 yo pt with new-onset iron deficiency anemia
colon cancer
microcytic anemia + swallowing difficulty + glossitis
Plummer-Vinson syndrome
microcytic anemia + >3.5% HbA2
B-thalassemia minor
megaloblastic anemia not correctable by B12 or folate
orotic aciduria
megaloblastic anemia along with peripheral neuropathy
B12 deficiency
microcytic anemia + basophilic stippling
lead poisoning
microcytic anemia reversible with B6
sideroblastic anemia
HIV-positive patient with macrocytic anemia
Zidovudine induced
Normocytic anemia and elevated creatinine
chronic renal disease
causes of hypochromic, microcytic anemias
iron deficiency, thalassemia, lead poisoning
hypersegmented neutrophils
megaloblastic anemia (B12 or folate)
skull x-ray shows a “hair-on-end” appearance
marrow hyperplasia
basophilic stippling of RBC
lead poisoning
findings associated with hereditary spherocytosis
anemia, jaundice, pigmented gallstones, splenomegaly, spherocytes
+ osmotic fragility test
(-) Coombs test
Ham’s test
Paroxysmal nocturnal hemoglobinuria
cells lyse when placed in acid (now use flow cytometry to look for no CD55/CD59)
Heinz bodies
G6PD deficiency
painful cyanosis of fingers and toes, with hemolytic anemia
cold autoimmune hemolytic anemia
red urine in the morning, and fragile RBC
paroxysmal nocturnal hemoglobinuria
basophilic nuclear remains in RBC
howell-jolly body
autosplenectomy
sickle cell disease
drug used to treat sickle cell disease
hydroxyurea
what does ADP and TxA2 do
ADP: induces GpIIb/IIIa expression on platelets
TxA2: promotes crosslinking of GpIIb/IIIa by fribrinogen
cause of ITP
Ab against GpIIb/IIIa
lab shows increased megakaryocytes
Bernard-Soulier disease
defective GpIb
mom bleeds profusely from vagina and later gums after delivery
DIC
DIC
increased BT, PT, PTT, D-dimers
decreased platelets and fibrinogen
STOP Making Thrombi
Sepsis, trauma, obstetrics, pancreatitis, malignancy, transfusions
antiplatelet antibodies
immune thrombocytopenia (ITP)
most common inherited bleeding disorder
von Willebrand disease
HUS-TTP
HUS triad: hemolysis (microangiopathic), renal insufficiency, thrombocytopenia
TTP adds neurologic symptoms and fever
TTP
defect in ADAMTS 13 that causes failure to breakdown vWF multimers
Glanzmann’s thrombasthenia
deficient GpIIb/IIIa
lab shows no platelet clumping
age distributions for Hodgkin and non-hodgkin lymphoma
Hodgkin peak at 20 and >65
non-Hodgkin variable but 20-40 common
most common non-Hodgkin lymphoma in adults and children
adult: diffuse large B cell lymphoma
children: lymphoblastic lymphoma
most common lymphoma in US
diffuse large B-cell
Reed-Sternberg cells
Hodgkin lymphoma
particularly associated with EBV
Burkitt, also Hodgkin lymphoma
associated with long-term celiac disease
intestinal
lymphoma equivalent of CLL
small lymphocytic lymphoma
“starry-sky pattern” due to phagocytosis of apoptotic tumor cells
Burkitt
associated with Sjogren, Hashimoto thyroiditis, and H. pylori
marginal cell MALToma
cancer most commonly associated with a noninfectious fever
Hodgkin lymphoma
sheets of lymphoid cells, with a “starry sky” appearance
Burkitt
large B cells with bilobed nuclei and prominent Owl’s eye inclusions
Hodgkin lymphoma
thyroid cells with optically clear nuclei
papillary carcinoma of thyroid
anemia with hypersegmented neutrophils
megaloblastic anemia
branching rods on oral infection
actinomyces israelii
eczema + recurrent infections + thrombocytopenia
Wiskott-aldrich syndrome
hemosiderinuria + thrombosis
paroxysmal nocturnal hemoglobinuria
CD30 and CD15+ B cells
Reed-sternberg cells in Hodgkin’s lymphoma
t(8:14)
Burkitt’s lymphoma
c-myc activation
t(14:18)
diffuse large B cell and follicular lymphoma
bcl-2 activation
t(11:14)
mantle cell lymphoma
cyclin D1 activation
caused by HTLV-1
adult T-cell lymphoma
t(9:22)
Philadelphia chromosome in CML
bcr-abl
t(15:17)
AML (pro-myelocytic)
responsive to all-trans retinoic acid
t(8:21)
AML in Down syndrome
anemia, hypercalcemia, and bone pain has BM biopsy that shows plasma cells
multiple myeloma
most common leukemia in children
ALL
most common leukemia in adults in US
CLL
characteristic Auer rods
AML
myelodysplastic syndromes have a tendency to progress to
AML
myeloproliferative disorders may progress to
AML
more than 20% blasts in marrow
acute leukemia
leukemia with more mature cells and <5% blasts
chronic leukemia
PAS (+) acute leukemia
ALL
commonly presents with bone pain
ALL
numerous basophils, splenomegaly, and negative for leukocytic alkalnine phosphate (LAP)
CML
always positive for philadelphia chromosome t(9:22)
CML
acute leukemia positive for peroxidase
AML
solid sheets of lymphoblasts in marrow
ALL
PAS - acute leukemia
AML
always associated with bcr-abl gene
CML
“smudge” cells
CLL
“punched out” lytic bone lesions
multiple myeloma
RBCs clumped together like a stack of coins
multiple myeloma
monoclonal antibody spike
multiple myeloma, monoclonal gammopathy of undetermined significance (MGUS), or Waldenstrom macroglobulinemia
reddish-pink rods in cytoplasm of leukemic blasts
Auer rods in AML
CD13/33 +
AML
may progress to AML or ALL
CML
95% have B cell markers
CLL
associated with Down syndrome
ALL and AML
associated with pancytopenia
Acute leukemia
pancytopenia
anemia
bleeding tendency
infection
multiple myeloma
think CRAB hyperCalcemia Renal insufficiency Anemia Back pain/lytic Bone lesions
auer rods: what genetic mutation
t(15:17)
AML