Term
| What anti-porter is on the surface of RBC membranes? |
|
Definition
Cl-HCO3 antiporter
Causes physiologic chloride shift that allows RBC to transport CO2 from periphery to lungs |
|
|
Term
| What are the things that bind to the vWF receptor and the Fibrinogen receptor? |
|
Definition
vWF = Gp1b
Fibrinogen = GpIIb/IIIa |
|
|
Term
| What enzymes are contained within PMNs? |
|
Definition
| hydrolytic enzymes, lysozyme, myeloperoxidase and lactoferrin |
|
|
Term
| In what condition do you see hypersegmented poly's? |
|
Definition
| Vit B12/Folate deficiency |
|
|
Term
| What activates macrophages? |
|
Definition
IFN-gamma
Released by TH1 cells |
|
|
Term
| What causes eosinophilia? |
|
Definition
NAACP
Neoplastic, Asthma, Allergic processes, Collagen vascular diseases, Parasites (invasive) |
|
|
Term
| What do mast cells release? |
|
Definition
histamine, heparin and eosinophil chemotactic factors
Can bind the Fc portion of IgE
Cromolyn sodium prevents mast cell degranulation |
|
|
Term
| What interleukins are involved in WBC differentiation? |
|
Definition
IL-3 at beginning
IL-11 involved in megakaryocyte differentiation |
|
|
Term
| What CD is present on all T cells? |
|
Definition
|
|
Term
| What are the deficiencies in Hemophilia A and B? |
|
Definition
A = factor VIII def
B = factor IX def |
|
|
Term
| What does antithrombin inhibit? What pathway does it inhibit? |
|
Definition
IIa, IXa, Xa, XIa and XIIa
Is activated by heparin
affects INTRINSIC pathway |
|
|
Term
| What factor does vWF carry and protect? |
|
Definition
|
|
Term
| What does activated protein C do? What activates it? What dz causes factor V to be resistant to this? |
|
Definition
Cleaves Va and VIIIa
Activated by protein S
Factor V Leiden |
|
|
Term
| What things produced by COX are pro-aggregtory and anti-aggretory? |
|
Definition
Pro-aggregation = TXA2 (thromboxane A2, released by platelets)
Anti-aggregation = PGI2 (prostacyclin) and NO (released by endothelial cells) |
|
|
Term
| What is Bernard-Soulier syndrome? Glanzmann's Thrombasthenia? |
|
Definition
Bernard-Soulier = def of GpIb
Glanzmann's = GpIIb/IIIa def |
|
|
Term
| What diseases are associated with basophilic strippling? |
|
Definition
BASte the ox TAIL
Thalassemia, Anemia of chronic dx, Iron def, Lead poisoning (all are MICROCYTIC except ACD = normocytic) |
|
|
Term
| What disease is associated with bite cells? |
|
Definition
|
|
Term
| What diseases are associated with acanthocytes? |
|
Definition
Liver disease and abetalipoproteinemia
spiny cells |
|
|
Term
| What causes ringed sideroblasts? |
|
Definition
| Sideroblastic anemia --> failure to completely synthesize heme so get Fe deposits in the mito that form a ring around the nucleus of a developing RBC |
|
|
Term
| What causes teardrop cells? |
|
Definition
Bone marrow infiltration (ex myelofibrosis)
"Bone marrow is crying b/c it is fibrosed" |
|
|
Term
| What causes Target cells? |
|
Definition
"HALT" said the hunter to his target
HbC disease, Asplenia, Liver disease, Thalassemia
Excess membrane relative to amount of Hb |
|
|
Term
| What are Howell-Jolly bodies? |
|
Definition
| Left over DNA, no spleen so can't expel it (usually leftover DNA is expelled by spleen) In asplenia or functional hyposplenia |
|
|
Term
| What are the causes of microcytic, hypochromic anemias? |
|
Definition
| Thalassemia, Fe deficiency, Lead poisoning (inhibit ferrochelatase or ALAD), Sideroblastic anemia (def ALAS, X-linked) |
|
|
Term
| What are the signs/symptoms of Lead poisoning? |
|
Definition
LEAD
Lead lines on gingivae and on epiphyses of long bones
Encephalopathy and Erythrocyte basophilic stippling
Abdominal colic and sideroblastic Anemia
Drops (wrist and foot drop)
Dimercaprol and EDTA for tx
Succimer for kids (it SUCKS to be a kid who eats lead) |
|
|
Term
| What are the causes of macrocytic anemia? |
|
Definition
Megaloblastic = folate/B12 def
Nonmegaloblastic = Liver dx, alcoholism, reticulocytosis, metabolic disorder, Drugs (5-FU, AZT, hydroxyurea)
Is stuff that interferes w/ DNA syn |
|
|
Term
| What are the neuro side effects of B12 deficiency? |
|
Definition
Peripheral neuropathy
Posterior columns
Lateral corticospinal
Dementia |
|
|
Term
| What does haptoglobin do? |
|
Definition
| Binds free Hb (so if hemolysis occurs intravascularly, will decrease haptoglobin b/c is binding Hb) |
|
|
Term
| Which type of hemolysis causes jaundice? |
|
Definition
Extravascular
hereditary spherocytosis, G6PD def, sickle cell |
|
|
Term
| What are the major causes of aplastic anemia? |
|
Definition
Radiation and drugs (benzene, chloramp, alkylating agents, antimetabolites)
Viral agents (B19, EBV, HIV, HCV)
Fanconi's anemia (defect in DNA repair)
Idiopathic, may follow acute hepatitis
I am not a FAN of RAD VIRUSES |
|
|
Term
| What are the antidotes for cyanide poisoning? |
|
Definition
| Nitrites and Sodium Thiosulfate |
|
|
Term
| How do androgens affect hematocrit? |
|
Definition
| Stimulate RBC production --> increased hematocrit |
|
|
Term
| What is the major cause of aplastic crisis in Sickle Cell patients? |
|
Definition
|
|
Term
| What can be used to treat Sickle Cell? |
|
Definition
| Hydroxyurea (increases HgF) |
|
|
Term
| What are the genetic mutations in Sickle Cell and HbC disease? |
|
Definition
Sickle Cell = Glutamate --> Valine
HbC = Glutamate --> Lysine |
|
|
Term
| Increased urine hemosidering and nocturnal darkening of urine |
|
Definition
Paroxysmal nocturnal hemoglobinuria
Impaired syn of GPI anchor --> decreased Decay Accelerating Factor |
|
|
Term
| What are the extrinsic hemolytic normocytic anemias? |
|
Definition
| Autoimmune hemolytic anemia (Warm and Cold agglutinins), Microangiopathic anemia (obstruction lumina), Macroangiopathic anemia (prosthetic heart valves, aortic stenosis) and Infections (malaria, babesia) |
|
|
Term
| What are warm agglutinins associated with? |
|
Definition
| SLE, CLL and some drugs (methyldopa) |
|
|
Term
| What are cold agglutinins associated with? |
|
Definition
| CLL, mycoplasma pneumoniae, infectious mononucleosis |
|
|
Term
| What is the cause of Idiopathic Thrombocytopenic Purpura? |
|
Definition
Anti-GpIIb/IIIa antibodies --> peripheral platelet destruction
increased megakaryocytes |
|
|
Term
| What is the cause of thrombotic thombocytopenic purpura? |
|
Definition
deficiency of ADAMTS 13 (vWF metalloprotease) so get decreased degradation of VWF multimers
Similar to HUS |
|
|
Term
| What does PT measure? PTT? |
|
Definition
PT = II, V, VII, X (extrinsic pathway)
PTT = everything BUT VII and XIII (intrinsic pathway) |
|
|
Term
| What are the effects of von Willebrand's disease? |
|
Definition
Nl or increased PTT
Lost VIII
Decreased vWF = defect in plt-to-collagen adhesion
AD! |
|
|
Term
| What will be the effect of Antithrombin III deficiency? |
|
Definition
| Reduced increased in PTT after administration of heparin |
|
|
Term
| What are the major characteristics of Hodgkin's Lymphoma? |
|
Definition
Reed-Sternberg cells
Contiguous spread of LN's
B signs/symptoms present (fever, night sweats, wt loss)
Mediastinal lymphadenopathy
50% cases associated w/ EBV
Good prognosis |
|
|
Term
| What are the major characteristics of Non-Hodgkin's lymphoma? |
|
Definition
Assoc w/ HIV and immunosupp
Multiple LN's, noncontinguous spread of LN's
Mostly B cells
Fewer constitutional signs/sx's
20-40 yo |
|
|
Term
| What are the CD's present on Reed-Sternberg cells? |
|
Definition
|
|
Term
| What are the types of Hodgkin's lymphoma? |
|
Definition
Nodular sclerosing - excellent prognosis, few RS cells, many lymphocytes
Mixed cellularity - intermed prognosis, same # RS and lymphocytes, assoc w/ EBV
Lymphocyte predominant - excellent prognosis, <35 yo males
Lymphocyte depleted - rare, poor prognosis |
|
|
Term
| What are the types of B cell Non-Hodgkin's Lymphoma? |
|
Definition
Burkitt's - t(8;14), c-myc, EBV
Diffuse large B-cell Lymphoma - most common, aggressive dissemination
Mantle Cell LYmphoma - t(11;14), poor prognosis, CD5+
Follicular lymphoma - t(14;18), bcl-2, difficult to cure w/ indolent course |
|
|
Term
| What are the types of T Cell Non-Hodgkin's Lymphoma? |
|
Definition
Adult T-cell lymphoma - HTLV-1, cutaneous lesions, esp in Japan, West Africa, and Caribbean, aggressive
Mycosis fungiodes/Sezary syndrome - cutaneous patches/nodules, indolent, CD4+ |
|
|
Term
| What are the symptoms of Multiple Myeloma? |
|
Definition
CRAB
hyperCalcemia, Renal insufficeincy, Anemia and Bony lytic lesions/Back pain |
|
|
Term
| What are the lab findings w/ Multiple Myeloma? |
|
Definition
Multiple Myeloma = Monoclonal M protein spike = IgM
Ig light chains in urine (bence jones)
Rouleaux formation (RBCs stacked like poker chips) |
|
|
Term
| How is Waldenstrom's macroglobulinemia differ from Multiple Myeloma? |
|
Definition
Both have M spike
Waldenstrom's has hyperviscosity symptoms and no lytic bone lesions
Is B cell neoplasm of plasmacytoid lymphocytes that produces monoclonal IgM
Hyperviscosity = retinal vascular dilatation, abnormal bleeding |
|
|
Term
| What is the translocation that is associated with a better prognosis of ALL? |
|
Definition
t(12;21) Remember Down's syndrome has increased risk of ALL ALL associated w/ kids!
Think Downs (tri 21) --> ALL t(12;21)
21!!! |
|
|
Term
| What are the findings w/ SLL/CLL? |
|
Definition
Asx
Smudge cells of pbs, warm Ab autoimmune hemolytic anemia
Cells are CD19+, CD20+, CD5+ and CD10-
Older adults (>60 yo) |
|
|
Term
| What are the findings w/ Hairy Cell leukemia? |
|
Definition
Mature B cell tumor
In ELDERLY
Stains TRAP positive |
|
|
Term
| What are the findings associated with AML? |
|
Definition
Auer rods!
FOunds in adults
APML = t(15;17) responds to ATRA! |
|
|
Term
| What are the findings associated with CML? |
|
Definition
Philadelphia chromosome = t(9;22), bcr-abl (codes for a tyr kinase)
Prominent splenomegaly
May accelerate to blast crisis (AML/ALL)
Very low alk phos (vs leukemoid rxn)
Responds to Imatinik (anti-bcr-abl Ab) |
|
|
Term
| What are the approximate ages of pts w/ ALL, AML, CML and CLL? |
|
Definition
ALL = <15
AML = ~60
CML = 30-60
CLL = >60 |
|
|
Term
| What is the translocation associated with Ewing's sarcoma? |
|
Definition
|
|
Term
What is the histiologic finding of Langerhans Cell Histiocytosis?
What do these cells express? |
|
Definition
Birbeck granules
S-100 and CD1a |
|
|
Term
| What are the chronic myeloproliferative disorders that are associated with JAK2 mutations? |
|
Definition
Poolycythemia vera, Essential thrombocytosis, Myelofibrosis
(CML assoc NOT with JAK2, but w/ philadelphia chromo) |
|
|
Term
| What diseases are associated with Polycythemia Vera? |
|
Definition
| RCC, Wilms' tumor, cyst, HCC, hydronephrosis |
|
|
Term
| Heparin and Warfarn affect which pathway of coagulation? |
|
Definition
Heparin = Intrinsic (PTT)
Warfarin = Extrinsic (PT/INR) |
|
|
Term
| Do Warfarin or Heparin cross the placenta? |
|
Definition
Warfarin = yes
Heparin = No |
|
|
Term
| What is the major side effect of Ticlopidine? |
|
Definition
|
|
Term
| How do Vinca Alkaloids and Taxanes affect microtubules? |
|
Definition
Vinca inhibits microtubule FORMATION
Taxanes inhibit mt disassembly
It's TAXING to stay polymerized! |
|
|
Term
| What alkylating agent can be used for Brain tumors (b/c it crosses the BBB)? |
|
Definition
| Nitrosoureas (carmustine, lomustine, semustine, streptozosin) |
|
|
Term
| What are the alkylating agents? |
|
Definition
| Cyclophosphamide, Nitrosoureas, Busulfan |
|
|
Term
| What are the toxicities of the vinca alkaloids? |
|
Definition
Vincristine = neurotox
Vinblastine = myelosuppression (BLASTs Bone marrow) |
|
|
Term
| What are the drugs that cross-link DNA? |
|
Definition
Cisplatin and Carboplatin
Nephrotoxicity and CN VIII damage |
|
|
Term
| What does hydroxyurea inhibit? |
|
Definition
| Ribonucleotide Reductase (decreased DNA synthesis) |
|
|
Term
| What makes Raloxifene different from Tamoxifen? |
|
Definition
| Raloxifene doesn't cause an increased in endometrial carcinoma b/c it is an endometrial antagonist |
|
|
Term
| What is the toxicity of Trastuzumab? |
|
Definition
Cardiotoxicity
(Herceptin) |
|
|
Term
|
Definition
Philadelphia chromosome bcr-abl tyrosine kinase inhibitor
For use in CML! |
|
|
Term
|
Definition
anti-CD20 antibody
For B-cell neoplasms
also RA (w/ methotrexate) |
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|
