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234 Cards in this Set

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a child was anemic since birth. In what disease would a splenectomy result in increased hematocrit?
spherocytosis
A person has macrocytic, megaloblastic anemia. Why should you not give folate alone?
It might be B12 deficiency. If you give folate, the symptoms improve, but nerve damage continues
Person has anemia, hypercalcemia, bone pain. Bone marrow cells are hypercellular with large nuclei. What's the dx, and what will be in the pee?
Multiple myeloma. You will likely see Bence-Jones protein in the urine
Which neoplasms are associated with AIDS?
B-cell lymphoma, Kaposi's sarcoma
What chemotherapeutic medicine should be avoided in patients with CHF?
doxyrubicin
what is the targeted therapy used to treat tumors with the Philadelphia chromosome (t(9:22)?
Imatinib (also called gleevec) inhibits the bcr-abl tyrosine kinase in these tumors
a 10-year old kid has a nonhealing ulcer on his leg and a small, calcified spleen. What drug could help this guy?
hydroxyurea, which increases fetal hemoglobin
Name the white blood cells in decreasing order of abundance in the body
Neutrophil, Lymphocyte, Monocyte, Eosinophil, Basophil
What does polycythemia mean?
Too many RBCs
A person with anisocytosis has.....?
varying sizes of RBCs
A person with poikilocytosis has.......?
varying shapes of RBCs
A person with reticulocytosis has.......?
too many immature RBCs
How long does an RBC live in the body?
120 days
What is the normal amount of leukocytes in each microliter of blood?
4,000-10,000
Which leukocyte has a butterfly-shaped nucleus and contains histamine, heparin, and LTD-4?
basophils
Which cell serves the same function as a basophil, but in the interstitium instead of the blood?
mast cells
Which leukocyte has a butterfly-shaped nucleus and uniform granules containing histamine and arylsulfate?
eosinophils
Which leukocyte defends against helminths and protozoans?
eosinophils
What 5 things will cause an increase in the number of eosinophils in the blood?
1) neoplasms
2) asthma
3) allergic processes
4) collagen vascular disease
5) parasites
NAACP
Which leukocyte has a multilobed nucleus and has granules containing lysozyme, myeloperoxidase, and lactoferrin?
neutrophils
What percentage of all white blood cells are neutrophils?
40-75%
Which leukocyte has a kidney-shaped nucleus, frosted glass cytoplasm, and is big?
Monocyte
What do monocytes differentiate into when they move into tissues?
macrophages
Which chemical activates macrophages?
gamma-interferon
Which cells are the best antigen-presenting cells in the body?
dendritic cells
What are dendritic cells called when they are in the skin?
langerhaans cells
What do lymphocytes look like?
big round nucleus, little cytoplasm
What is MHCII and what does it do?
a protein on the surface of monocytes, macrophages, dendritic cells, and B cells. It presents antigen to T-cells
what do plasma cells look like?
Off-center nucleus with spotty chromatin, lots of ER and golgi
Which lymphocyte has CD 19 and CD20?
B cells and plasma cells
Which lymphocyte has CD3 and CD4 on its surface?
Helper T cells
Which lympcyte has CD3 and CD8 on its surface?
cytotoxic T cells
What percentage of circulating lymphocytes are T cells?
80%
Where do B cells live in the spleen?
White pulp
Where do B cells live in the lymph nodes?
follicle
Name the factors in order in the Intrinsic Coagulation Cascade
12 ->11 -> 9, 9+8 -> 10, 10+5 -> prothrombin ->fibrinogen ->clot formation
Name the factors in order in the Extrinsic Coagulation Cascade
tissue factor + 7 ->10, 10+5 -> prothrombin ->fibrinogen -> clot formation
Which coagulation factors need vitamin K?
prothrombin, 7, 9, 10, protein C, and protein S
What does protein C do?
inactivates factors 5 and 8
What turns on antithrombin?
heparin
What does antithrombin do?
inactivates thrombin, 9, 10, and 11
Which chemical activates plasminogen into plasmin?
tPA
What does plasmin do?
cleaves fibrin, breaks up clots
What does factor 12 activate besides factor 11?
the bradykinin system
What does the bradykinin system do?
increase vasodilation
increase vessel permeability
increase pain
What chemical is required for platelets to stick to exposed basement membrane?
von willebrand factor
Which two chemicals decrease platelet aggregation?
prostacyclin and NO
what chemical increases platelet aggregation?
thromboxane
Which blood type is the universal recipient?
AB. A and B antibodies on cells, no antibodies in plasma
Which blood type is the universal donor?
O. no antibodies on the cells, both antibodies in the plasma
Why are normal RBCs shaped the way they are?
The bi-concave shape maximizes surface area for a given volume; better oxygen off-loading
What disease results in spherocyte RBCs?
Hereditary spherocytosis. Due to a defect in a structural protein
What disease results in elliptocyte RBCs?
hereditary elliptocytosis
What disease results in macro-ovalocyte RBCs?
megaloblastic anemia (B12 and folate deficiency), marrow failure
What disease results in helmet cells and schistocyte RBCs?
DIC, traumatic hemolysis
What disease results in sickle-shaped RBCs?
Sickle cell anemia
What disease results in bite cell RBC's?
G6PD deficiency
What disease results in teardrop shaped RBCs?
myeloid metaplasia with myelofibrosis
What disease results in acanthocyte RBCs?
abetalipoproteinemia. acanthocytes are spiky
What disease results in target RBC's?
HbC disease, Asplenia, Liver disease, Thalassemia
What disease results in poikilocyte RBCs?
TTP/HUS, microvascular damage, DIC
What disease results in burr cell RBCs?
TTP/HUS
(thrombotic thrombocytogenic purpura / hemolytic uremic syndrome)
What disease process results in basophilic stippling?
Thalassemia, anemia, iron deficiency, lead poisoning
What disease can cause hypersegmented PMNs?
folate/B12 deficiency
What 4 things cause microcytic, hypochromic anemia?
1)iron deficiency
2)thalassemia
3)lead poisining
4)sideroblastic anemia (malignancy)
What is the MCV in a person with microcytic anemia?
<80
What are the 3 causes of macrocytic anemia?
1)megaloblastic (lack folate/B12)
2)drugs that block DNA synthesis
3)marked reticulocytosis
What is the MCV in a person with macrocytic anemia?
>100
A person with decreased serum haptoglobin and increased serum LDH likely has what process going on?
anemia due to RBC hemolysis
What test is used to distinguish autoimmune RBC hemolysis with other forms of hemolysis?
the coombs test
A person has macrocytic anemia and neurological deficits. What is the problem?
They are deficient in vitamin B12
G6PD and PK deficiency result in what kind of anemia?
normocytic, normochromic
Acute hemorrhage results in what type of anemia?
normocytic, normochromic
Anemia due to iron deficiency. What are the lab values for serum iron, transferrin/TIBC, ferritin, %transferrin saturation?
serum iron: low
transferrin/TIBC: high
ferritin: low
%transferring sat: very low
Anemia due to chronic disease. What are the lab values for serum iron, transferrin/TIBC, ferritin, %transferrin saturation?
serum iron: low
transferrin/TIBC: low
ferritin: high
%transferrin sat: unchanged
Anemia due to pregnancy/OCP use. What are the lab values for serum iron, transferrin/TIBC, ferritin, %transferrin saturation?
serum iron: unchanged
transferrin/TIBC: high
ferritin: unchanged
%transferrin sat: low
Anemia due to hemochromotosis. What are the lab values for serum iron, transferrin/TIBC, ferritin, %transferrin saturation?
serum iron: high
transferrin/TIBC: low
ferritin: high
%transferrin sat: very high
What happens in aplastic anemia?
destruction of the myeloid stem cell in bone marrow leads to marked pancytopenia
What causes aplastic anemia?
radiation, benzene, viruses, alkylating agents, chloramphenicol, idiopathic
what are the symptoms of aplastic anemia?
fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infections
In a person with with aplastic anemia, what do the blood cells look like?
Cells look normal, there are just not very many of them
How would you treat a person with aplastic anemia
marrow transplant, blood transfusion, stop giving them the offending agent
What is mutated in sickle cell disease?
beta chain in hemoglobin (glutamic acid replaced with a valine)
In general, what is the problem in alpha-thalassemia?
underproduction of the alpha chain in hemoglobin
In general, what is the problem in beta-thalassemia?
underproduction of the beta chain in hemoglobin
What stimulates sickling in a person with sickle cell disease?
Low O2, dehydration
What is the treatment for sickle cell disease?
bone marrow transplant, hydroxyurea (to increase HgF), supportive treatments
What are some of the complications of sickle cell disease?
aplastic crisis, autosplenectomy, infections, vaso-occlusive crisis, renal papillary necrosis, splenic sequestration
In general, what happens in a person with HgC disease?
also a beta chain mutation, but not as serious as sickle cell
What is the difference between beta-thal major and beta-thal minor>
beta-thal minor = beta chain is present, but underproduced
beta-thal major = beta chain is completely absent
Which thalassemias result in intrauterine fetal dealth?
HbH and Hb Barts, both of which are alpha thalassemias
What does the body compensate with in alpha-thalassemia?
nothing, all hemoglobins need alpha chains, so there is no compensatory mechanism
What does the body compensate with in beta-thalassemia?
increased HbF
All hemolytic anemias present with what two signs?
increased serum bilirubin (jaundice) and increased reticulocytosis
What is the procedure for a direct Coomb's test?
add anti-Ig antibodies to patient's RBCs. If RBC's agglutinate, then the test is positive
What is the procedure for an indirect Coomb's test?
normal RBC's added to patient's serum. If RBCs agglutinate, then the test is positive
In autoimmune hemolytic anemia, where are the RBCs destroyed?
extravascularly, so, in the liver and spleen
What happens in Warm Autoimmune hemolytic anemia?
IgG attacks RBCs in warm temperatures. Seen in lupus, CLL, or with drugs
What happens in Cold Autoimmune hemolytic anemia?
IgM attacks RBCs in cold temperatures. Seen in mono or mycoplasmic pneumonias
What happens in erythroblastosis fetalis?
seen in newborns when their blood Rh doesn't match the mom's Rh, and the mom's antibodies attack the baby's RBCs
What happens in hereditary spherocytosis?
defect in ankyrin or spectrin causes RBCs to be balls instead of disks.
How do you diagnose hereditary spherocytosis?
spherocytes on blood smear, negative coomb's test, osmotic fragility test
What happens in paroxysmal nocturnal hemoglobinemia?
membrane defects lead to complement-induced intravascular hemolysis
What happens in microangiopathic anemia, and what are its 4 causes?
intravascular hemolysis due to DIC, lupus, TTP/HUS, or malignant hypertension
In general, what happens during DIC?
activation of coagulation cascade, leads to tons of micro-thrombi that use up all the platelets, fibrin, and co-factors
What are the 7 causes of DIC?
sepsis, trauma, obstetric problems, pancreatitis, malignancy, nephrotic syndrome, transfusion
What will lab findings be in DIC?
elevated PT and PTT, high fibrin split products, high D-dimer, low platelet count
What happens in Idiopathic Thrombogenic Purpura (ITP)?
peripheral platelet destruction, antiplatelet antibodies, increased megakarycytes, microbleeds, bruising
What happens in Thrombotic Thrombogenic Purpura (TTP)?
platelet aggregation, thrombosis, high LDH, neuro and renal symptoms, fever
What 5 things cause bleeding disorders due to platelet anomalies?
1)ITP
2)TTP
3)DIC
4)aplastic anemia
5)medications
What 3 things cause bleeding disorders due to coagulation cofactor anomalies?
1)Hemophilia A
2)Hemophilia B
3)von Willebrand disease
All bleeding disorders due to platelet anomalies present with:
microhemorrhage, mucous membrane bleeding, petechiae, purpura, nosebleeds
All bleeding disorders due to coagulation cofactor anomalies present with:
macrohemorrhage, bleeding into joints, easy bruising, high PT or PTT
A person who is deficient in Factor 8 has.....?
Hemophilia A
A person who is deficient in Factor 9 has......?
Hemophilia B
What happens in a person who has von Willebrand's disease?
lack von Willebrand Factor, which binds platelets to exposed basement membrane. mild disease
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in Thrombocytopenia?
platelet count: down
bleeding time: up
PT: unchanged
PTT: unchanged
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in Hemophilia A?
platelet count: unchanged
bleeding time: unchanged
PT: unchanged
PTT: high
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in Hemophilia B?
platelet count: unchanged
bleeding time: unchanged
PT: unchanged
PTT: high
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in von Willebrand's Disease?
platelet count: unchanged
bleeding time: high
PT: unchanged
PTT: high
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in DIC?
platelet count: low
bleeding time: high
PT: high
PTT: high
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in Vitamin K deficiency?
platelet count: unchanged
bleeding time: unchanged
PT: high
PTT: high
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in Bernard-Soulier Disease?
platelet count: low
bleeding time: high
PT: unchanged
PTT: unchanged
platelet count, bleeding time, PT, and PTT: high, low, or unchanged in Glanzmann's thrombasthenia?
platelet count: unchanged
bleeding time: high
PT: unchanged
PTT: unchanged
Where is the defect in Bernard-Soulier Disease?
defect in platelet adhesion
Where is the defect in Glansmann's thrombasthenia?
defect in platelet aggregation
Which co-factors does the PT time measure?
the extrinsic pathway, cofactors 5, 7, 10, and thrombin
Which co-factors does the PTT time measure?
the intrinsic pathway, every cofactor except 7
Which disease is characterized by the appearance of Reed-Steenberg cells?
Hodgkin's Disease
What do you call a giant cell seen in a tumor, with a bilobed nucleus that looks like owl's eyes?
Reed-Steenberg cell
What normal cell does a Reed-Steenburger cell arise from?
B cell
Which type of lymphoma spreads contiguously from a single, localized source?
Hodgkin's Lymphoma
Which type of lymphoma appears in many nodes at once, and does not spread contiguously?
Non-Hodgkin's Lymphoma
hypergammaglobinemia is a sign for which type of lymphoma?
Hodgkin's lymphoma
which lymphoma does not present with fever, night sweats, or weight loss?
non-Hodgkin's lymphoma
Which virus is implicated in stimulating Hodgkin's lympoma?
Epstein Barr Virus (EBV)
Which lymphoma is associated with HIV and immunosuppression?
non-Hodgkin's lymphoma
Which lymphoma presents in people 20-40 years old?
non-Hodgkins lymphoma
Which lymphoma presents in people under 20, or over 40?
Hodgkins Lymphoma
Nodular sclerosing Hodgkin's Lymphoma is characterized by what?
collagen banding and lacunar cells in lymph nodes, few Reed-Steenberg cells, lots of normal lymphocytes
What is the biggest factor in deciding the prognosis of a person with Hodgkin's Lympnoma?
Reed-Steenberg cells to lymphocytes ratio; the more RS cells compared to lymphos, the worse the prognosis
Where is the tumor in Multiple Myeloma?
In the bone marrow
In multiple myeloma, what is the precursor cell to the tumorous cell?
Plasma cell
in Multiple Myeloma, what do the cancerous plasma cells look like?
fried eggs, apparently.
In multiple myeloma, what do the tumors secrete?
Tons of IgG and IgA
What are Benze-Jones protein?
Ig light chains in the urine
What are 6 of the symptoms of multiple myeloma?
hypercalcemia, renal insufficiency, infections, anemia, amyloidosis, lytic bone lesions
What are 3 lab results in a person with Multiple Myeloma?
Benze-Jones protein in urine, M-spike on serum protein electrophoresis, RBCs stacked like poker chips on blood smear
Of the non-hodgkins lymphomas, which one is cancer of T cells?
Lymphoblastic Lymphoma
what non-hodgkin's lymphoma presents in children with "starry sky" histology and is associated with EBV?
Burkitt's Lymphoma
Which gene translocation results in CML (chronic myelongenous leukemia)?
t(9:22)
the Philadelphia chromosome
Which genetic transposition results in Burkitt's Lymphoma, and what gene does it activate?
t(8:14)
c-myc is activated
Which genetic transposition results in Follicular Lymphomas, and what gene does it activate?
t(14:18)
bcl-2 is activated
Which genetic transposition results in AML (acute myelogenous leukemia)?
t(15:17)
What drug do you use to treat Acute Myelogenous Leukemia (AML)?
trans-retinoic acid, otherwise known as Vitamin A
Which genetic transposition results in Ewing's Sarcoma?
t(11:22)
Which genetic transposition results in Mantle Cell Lymphoma in adults?
t(11:14)
What two things comprise the Leukemoid Reaction?
increased band cells and increased levels of leukocyte alkaline phosphatase
In general, what goes on in a person with leukemia?
neoplasm in bone marrow. high abnormal WBCs, low RBCs, low platelets, infection, anemia, bleeding
What population does ALL (acute lymphoblastic leukemia) usually present in?
children
What are the precursor cells to the neoplasms in ALL?
lymphoblasts (immature B and T cells)
Which cells are the precursors to the neoplasm in AML?
myeloblasts (leukocyte pecursors)
What are Auer Rods and where do they appear?
needle-shaped clumps of granule material in the neoplastic myeloblast cells in AML
Which population does CLL (chronic lymphoblastic leukemia) usually present in?
Older adults, those over 60
What 4 signs are associated with CLL?
lymphadenopathy, hepatosplenomegaly, warm antibody anemia, smudge cells on peripheral smear
What 3 signs are associated with CML?
increased neutrophils, increased metamyelocytes, splenomegaly
What is Hairy Cell Leukemia, and who does it affect?
Mature B cell tumor, it affects old people
What condition is caused by LangerHaans cells infiltrating the lungs in young adults?
Histiocytosis X
what is the mechanism of Warfarin?
activates antithrombin, decreases thrombin and cofactor 10
Does Heparin affect the PT or the PTT?
The PTT
What drug rapidly neutralizes heparin?
Protamine sulfate
Between Warfarin and Heparin, which can you give a pregnant woman?
Heparin, it does not cross the placenta
What happens during Heparin-Induced Thrombocytopenia?
heparin binds platelets, causing antibodies to destroy those and over-activate others. results in hypercoagulable state
How do Lepirudin and Bivalirudin work?
directly bind to thombin and inactivates it
How does Warfarin work?
shuts down gamma-carboxylation of Vit-K dependant co-factors 7, 9, 10, thrombin, protein C and S
Which pathway does Warfarin affect, and does it affect PT or PTT?
Warfarin affects the extrinsic pathway, and affects PT
How do thrombolytic drugs work?
turn plasminogen into plasmin, which dissolves clots and inactivates thrombin
When do you use thrombolytic drugs?
early acute MI or early stoke. All other uses, the risk of bleeding is too great
How do thrombolytic drugs affect PT, PTT, and platelet count?
increase PT, increase PTT, no change in platelet count
What surface protein on a platelet binds to other platelets during clot formation?
GP2b/3a
How does aspirin affect platelets?
decreased thromboxane production by inhibiting COX1 and COX2
What do ticlopidine and clopridogrel do?
prevent GP2b/3a expression on platelet surfaces, so they have nothing to bind to
What does abcixamab do?
antibody that binds to GP2b/3a so that platelets are unable to aggregate
What does methotrexate do?
blocks the synthesis of thymidine, used to treat cancer
What does fluorouracil (5-FU) do?
blocks the synthesis of thymidine and other pyramidines, used to treat cancer
What does mercaptopurine (6-MP) do?
blocks the synthesis of purines, used to treat cancer
What does cytarabine do?
Damages DNA during the S-phase in cells. Used to treat cancer
What does cisplatin do?
Crosslinks DNA, causes cell apoptosis. Used to treat cancer
What does dactinomycin do?
Blocks RNA polymerase. Used to treat cancer
What does doxorubicin do?
intercalates between DNA base pairs. Used to treat cancer
What does bleomycin do?
cleaves DNA strands. Used to treat cancer
What does etoposide do?
Inhibits topoisomerase 1, leads to DNA strand breaks. used to treat cancer
What does tamoxifen do?
selectively blocks estrogen receptors. Used to treat breast cancer
what do vinca alkaloids do?
inhibit microtubule formation, leading to decreased mitosis. Used to treat cancer
What does paclitaxel do?
Inhibits microtubule breakdown, leading to decreased mitosis. Used to treat cancer
Paclitaxel works best during which phase of the cell cycle?
Mitosis
Vinca alkaloids work best during which phase of the cell cycle?
Mitosis
Bleomycin works best during which phase of the cell cycle?
G2
etoposide works best during which phase of the cell cycle?
S and G2
Methotrexate works best during which phase of the cell cycle?
S
Fluorouracil works best during which phase of the cell cycle?
S
Which phase of the cell cycle does mercaptopurine work best in?
S
What phase of the cell cycle does cisplatin work best in?
doesn't matter, works all the time
which phase of the cell cycle does doxorubicin work best in?
doesn't matter, works in all of them
which phase of the cell cycle does dactinomycin work best in?
doesn't matter, works all the time
What do the nitrosourea drugs do?
alkylate DNA. used to treat cancer
What does busulfan do?
alkylates DNA. Used to treat cancer
What does hydroxyurea do?
inhibits ribonucleotide reductase, decreases DNA synthesis. Used to treat cancer
What phase of the cell cycle does hydroxyurea work best in?
S
How does prednisone work in treating cancer?
stimulates apoptosis
What does trastuzumab do? (herceptin)
antibody that binds to HER-2 receptors. Used to treat breast cancer
What does imatinib do? (gleevec)
antibody to bcr-abl tyrosine kinase that shows up due to the philadelphia chromosome
these two drugs are vinca alkaloids:
vincristine, vinblastine
these 4 drugs are nitrosoureas:
carmustine, lomustine, semustine, streptozocin
What are 3 side effects of methotrexate?
myelosuppression (fix with folinic acid), fatty change in liver, mucositis
What are 2 side effects of fluorouracil?
myelosuppression (cannot fix with folinic acid), photosensitivity
What are 3 side effects of cytarabine?
leukopenia, thrombocytopenia, megaloblastic anemia
What are 2 side effects of cyclophosphamide?
myelosuppression, hemorrhagic cystitis
Which organs does mercaptopurine adversely affect?
Liver, GI tract, bone marrow
what is 1 side effect of the nitrosoureas?
CNS toxicity (dizziness, ataxia)
What are 2 side effects of cisplatin?
nephrotoxicity and acoustic nerve damage
What are 2 side effects of busulfan?
pulmonary fibrosis, hyperpigmentation
What are 3 side effects of doxorubicin?
cardiotoxic, myelosupression, alopecia
What is 1 side effect of dactinomycin?
myelosuppression
what are 2 side effects of bleomycin?
pulmonary fibrosis, skin changes. NOT myelosuppression, oddly enough
What are 2 side effects of hydroxyurea?
bone marrow suppression, GI upset
what are 3 side effects of etoposide?
myelosupression, GI irritation, alopecia
what 1 side effect of tamoxifen?
increased risk of endometrial carcinoma
What is 1 side effect of trastuzumab?
cardiotoxicity
What is 1 side effect of imatinib?
fluid retention
What are 2 side effects of vincristine?
neurotoxicity, paralytic ileus
What is 1 side effect of vinblastine?
bone marrow suppression
What are 2 side effects of paclitaxel?
myelosuppression and hypersensitivity
Teardrop RBCs and a large spleen suggest which disease?
adenogenic myeloid metaplasia