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104 Cards in this Set

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source of energy for erythrocytes?
glucose (90% anaerobically degraded to lactate; 10% by HMP shunt)
densely basophilic granules containing heparin, histamine & other vasoactive amines, and leukotrienes (LTD-4)
basophil
where are mast cells found?
tissue
cells that mediate allergic reaction?
mast cells and basophils
What prevents mast cell degranulation?
cromolyn sodium
cells that defend against helminthic and protozoan infections?
eosinophils
causes of eosinophilia?
"NAACP:
neoplastic, asthma, allergic processes, collagen vascular diseases, parasites"
when are hypersegmented neutrophils seen?
vitamin B12 deficiecncy and Folate deficiency
Which cells contain large, spherical, azurophilic primary granules that contain hydrolytic enzymes, lysozyme, myeloperoxidase, and lactoferrin
neutrophils
kidney-shaped nucleus; extensive 'frosted glass' cytoplasm; differentiates into macrophages in tissues
monocyte
off-center nucleus, clock-face chromatin distribution, abundant RER and well-developed Golgi
plasma cell
multiple myeloma is what type of neoplasm?
plasma cell
which cells are the main inducers of the primary antibody response?
dendritic cells
what is the role of tPA?
generates plasmin, which cleaves fibrin
what does PTT measure?
intrinsic pathway
what does PT measure?
extrinsic pathway - factors II, V, VII, X
what initiates the intrinsic pathway?
factor XII
what initiates the extrinsic pathway?
factor VII
what do proteins C and S inactivate?
Va and VIIIa; dependent on vitamin K
waht does antithrombin III inactivate?
thrombin, IXa, Xa, XIa; activated by heparin
what usually has more prognostic value - stage or grade?
stage
degree of cellular differentiation based on histologic appearance of tumor?
grade
degree of localization/spread based on site and size of primary lesion, spread to regional lymph nodes, presence of metastases, etc.
stage (S-spread)
macro-ovalocytes are seen in what?
megaloblastic anemia (also hypersegmented PMNs), marrow failure
helmet cells and schistocytes are seen when?
DIC, traumatic hemolysis
teardrop cells are seen when?
myeloid metaplasia with myelofibrosis
acanthocytes are seen when
spiny appearance in abetalipoproteinemia
when are target cells seen?
HALT: HbC disease, asplenia, liver disease, thalassemia
what are poikilocytes & when are they seen?
nonuniform shapes in TTP/HUS, microvascular damge, DIC
when are Burr cells seen?
TTP/HUS
etiologies of microcytic, hypochromic anemia?
iron deficiency, thalassemias, lead poisoning, sideroblastic anemias
etiologies of macrocytic anemia?
megaloblastic - vitamin B12/folate deficiency
drugs that block DNA synthesis (e.g. sulfa drugs, phenytoin, AZT)
marked reticulocytosis (bigger than mature RBCS)
causes of normocytic, normochromic anemia?
acute hemorrhage, enzyme defects (G6PD, PK), RBC membrane defects (e.g. hereditary spherocytosis), bone marrow disorders (aplastic anemia, leukemia), hemoglobinopathies (sickle cell), anemia of chronic disease, AI hemolytic anemia
what do decreased serum haptoglobin and increased serum LDH indicate?
RBC hemolysis
causes of aplastic anemia?
radiation, benzene, chloramphenicol, alkylating agents, antimetabolites, viral agents (parvovirus B19, EBV, HIV), Fanconi's anemia, idiopathic; may follow acute hepatitis
symptoms of aplastic anemia?
fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection
pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration.
What is the diagnosis?
aplastic anemia
what is the HbS mutation?
single AA replacement in beta chain - substitution of normal glutamic acid with valine
therapies for sickle cell anemia?
hydroxyurea (increases HbF), bone marrow transplant
what percent of blacks carry the Hb trait? what percent have the disease?
8%, 0.2%
crew cut on skull x-ray?
sickle cell anemia, thalassemias - due to marrow expansion from increased erythropoeisis
what is the defect in beta thalassemia minor? major?
minor - heterozygote - beta chain is underproduced
major - homozygote - beta chain is absent
why does cardiac failure occur in thalassemia?
secondary hemochromatosis
patient has marked erythrocytosis, moderate increase in granulocytes and platelets, splenomegaly, decreased EPO.
What is the diagnosis?
polycythemia vera
labs in anemia of chronic disease?
decreased TIBC, increased ferritin, low serum iron
findings in hemolytic anemia?
increased serum unconjugated bilirubin, increased reticulocytes (marrow compensating for anemia)
test for Autoimmune hemolytic anemia?
Coombs positive
chronic anemia seen in SLE, CLL, or with certain drugs (e.g. alpha methyldopa)
warm agglutinin (IgG)
describe the direct Coombs' test
anti-Ig Ab added to patient's RBCs agglutinate if RBCs are coated with Ig
Describe the indirect Coombs' test
normal RBCs added to patient's serum agglutinate if serum has anti-RBC surface Ig
microvascular hemolysis seen in DIC, TTP/HUS, SLE, or malignant hypertension describes what disease?
microangiopathic anemia
what is seen on blood smear in microangiopathic anemia?
schistocytes (helmet cells)
what is increased in paroxysmal nocturnal hemoglobinuria?
urine hemosiderin
activation of coagulation cascade leading to microthrombi and global consumption of platelets, fibrin, and coagulation factors
DIC
lab findings in DIC?
increased PT, PTT, fibrin split products (D dimers); decreased platelet count
what is seen on blood smear in DIC?
helmet-shaped cells and schistocytes
antiplatelet antibodies coating and damaging platelets, increased megakaryocytes, children
ITP
hyaline microthrombi in small vessels, helmet cells and schistocytes, neurologic abnormalities and renal insufficiency.
What is the diagnosis?
TTP
microhemorrhage - mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time. What is the underlying defect and differential diagnosis?
platelet abnormalities - ITP, TTP, DIC, aplastic anemia, drugs (e.g. immunosuppressive agents)
factor VIII deficiency
hemophilia A
factor IX deficiency
hemophilia B
describe von Willebrand's disease
mild; most common bleeding disorder; deficiency of vWF - defect of platelet adhesion and decreased factor VIII survival
macrohemorrhage - hemarthroses (bleeding into joints), easy bruising, increased PT, PTT. What is the category of disease and differential diagnosis?
coagulation factor deficiencies; Hemophilia A, Hemophilia B, von Willebrand's disease
Bernard-Soulier disease?
defect of platelet adhesion (decreased GP Ib) - prolonged bleeding time and decreased platelets
Glanzmann's thrombasthenia
defect of platelet aGgregation - decreased GP IIb-IIIa - prolonged bleeding time
decresed platelet count, increased bleeding time with normal PT and PTT. Diagnosis?
thrombocytopenia
or Bernard Soulier's
lab findings in hemophilia A or B?
normal platelet count, bleeding time, PT, increased PTT
increased bleeding time and increased PTT
von Willebrand's disease
findings in DIC
decreased platelet count, increased bleeding time, PT, and PTT
PT measures what?
extrinsic pathway - factors, II, V, VII, X
PTT measures what?
intrinsic pathway - all factors except VII
What are common complications associated with Sickle Cell Disease?
aplastic crisis (e.g. from parvovirus B19), autosplenectomy (occurs by 20 y/o), salmonella osteomyelitis, renal papillary necrosis, splenic sequestration crisis
What are common causes of DIC?
STOP Making New Thrombi
Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion
What causes Paroxysmal Nocturnal Hemoglobinuria?
Intravascular hemolysis due to membrane defect that increases sensitivity of RBCs to lytic activity of complement
(GPI anchor deficiency)
What is Cold agglutinin?
Where is it seen?
IgM mediated acute anemia triggered by cold. Seen in M. pneumoniae infections or infectious mononucleosis
What causes Hereditary Spherocytosis?
intrinsic extravascular hemolysis due to defect in spectrin or ankyrin
patient has increased MCHC, increased RDW, and RBCs with no central pallor. What is the diagnosis?
Hereditary spherocytosis
What cytokine activates macrophages?
interferon gamma
What proteins are expressed on the surface of dendritic cells?
MHC II and Fc receptors
Cytotoxic T lymphocytes are of which cluster designation and MHC complex?
CD4 and MHC II
Helper T cells are of which cluster designation and MHC complex?
CD8 and MHC I
Which are the vitamin K dependent factors?
II, VII, IX, X and proteins C and S
What activates protein C?
Thrombomodulin (protein S is a cofactor)
What is caused by a factor V Leiden mutation?
resistance to activated protein C
What do eosinophils produce?
histaminase and aryl sulfatase
When are bite cells seen?
G6PD deficiency
In what conditions would you see basophilic stippling?
TAIL - Thalassemia, Anemia of chronic disease, Iron deficiency, Lead poisoning
How do you distinguish between B12 and folate deficiencies clinically?
B12 deficiencies have neurologic manifestations
How do you diagnose aplastic anemia?
Bone Marrow biopsy
patient comes in with normal platelet count, normal bleeding time, normal PT and increased PTT. What is the diagnosis?
Hemophilia A or B
Patient presents with normal platelet count, increased bleeding time, normal PT and increased PTT. What is the diagnosis?
von Willebrand's disease
Patient has decreased platelet count, increased bleeding time, PT and PTT. What is the diagnosis?
DIC
patient has normal platelet count and bleeding time but increased PT and PTT. What is the diagnosis?
Vitamin K deficiency
patient has decreased platelet count and increased bleeding time with normal PT and PTT. What is the diagnosis and mechanism?
Bernard-Soulier disease;
decreased GP Ib (platelet adhesion)
Patient has increased bleeding time and normal platelet count, PT and PTT. What is the diagnosis and mechanism?
Glanzmann's thrombasthenia;
defect of GPIIb-IIIa (aggregation)
patient has decreased serum Iron, increased transferrin/TIBC, decreased Ferritin and decreased %transferrin saturation. What is the diagnosis?
iron deficiency
Patient has decreased serum iron, decreased transferrin/TIBC, increased ferritin and normal % transferrin saturation. What is the diagnosis?
anemia of Chronic disease
patient has normal serum iron, increased transferrin/TIBC, normal Ferritin and decreased % transferrin saturation. What is the diagnosis?
Pregnancy/OCP use
patient has increased serum iron, decreased transferrin/TIBC, increased Ferritin and markedly increased % transferrin saturation. What is the diagnosis?
hemochromatosis
What are the 3 steps of the formation of a platelet plug?
1. Platelet adhesion to exposed BM
2. Aggregation
3. Swelling - ADP and Ca release to strengthen plug and induce fibrin deposition
How is platelet aggregation regulated in clot formation?
1. TXA2 is released by platelets in induce aggregation
2. PGI2 and NO are released by endothelial cells to inhibit aggregation
PTT measures function of which clotting factors?
all factors except VII (intrinsic pathway)
PT measures function of which clotting factors?
II, V, VII and X (extrinsic pathway)
bilobed nucleus, densely basophilic granules containing heparin, histamine and other vasoactive amines and leukotrienes. found in blood. what is the cell?
Basophil