First Aid pg 397-401 Heme Flashcards
What causes cell death due to peroxidation of membrane lipids?
Fe poisoning
Symptoms of Fe poisoning?
n/v, gastric bleeding, lethargy, scarring leading to GI obstruction
Drugs used for Fe poisoning?
Chelation - IV deferoxamine, oral deferasirox
Which factors are tested in PT?
Factors I, II, V, VII, and X
Which factors are not tested in PTT?
All factors except VII and XIII
Inheritance patterns of all three hemophilias?
Hemophilia A and B - XLR, and C is AR
Tx for Hemophilia A?
Desmopressin + factor VIII concentrate
Vitamin K def will have an increase in what lab tests?
PT and PTT, Bleeding time is Normal
What parts of the coag pathway are affected by Vit K def?
F II, VII, IX, X, and Prot C and S
Which 3 hemat diseases have an issue with platelet plug formation?
Both Bernard Soulier, Glanzmann thrombasthenia, vW disease
Which platelet disorder has normal platelet count?
Glanzmann , vW disease
With which heme disorder will you potentially see enlarged platelets?
Bernard Soulier - release of relatively immature platelets to replace loss.
Which heme disorder is associated with inc BUN ?
TTP, DIC, Hemolytic Uremic Syndrome, PNH, Sickle Cell, porphyria, Multple Myeloma
What enzyme activates vit K?
vit K epoxide reductase
Normal bleeding time?
2-7 min
Normal PT time?
11-15 sec
Normal PTT time?
25-40 sec
Which two hematological disorders affect GpIIb/IIIa? How do they effect it?
Glanzmann - dec levels of GpIIb/IIIa
Immune Thrombocytopenia - anti GpIIb/IIIa antibodies
Which hemat disorder show inc megakaryocytes on bone marrow biopsy?
ITP
Which hemat disorder has a def of metalloprotease?
TTP - ADAMTS 13 = vWF metalloprotease
Schistocytes are seen in which platelet disorder? why?
TTP - due to microthrombi formation (inc vWF multimers –> Inc platelet adhesion –> Inc platelet aggregation and thrombosis) DIC, HUS
Which two hemat disorders have neurological symptoms?
TTP and HUS - pentad of neuro/renal symptoms, fever, thrombocytopenia, fever, microangiopathic hemolyic anemia
What is the difference between HUS in adults and children?
Children tend to have diarrhea, adults do not.
Most common cause of dec platelet #?
ITP
Which blood tests are elevated in vW disease?
BT inc, and PTT may be normal or Inc
Explain the Ristocetin test result of vW Disease?
No platelet aggregation, without vW.
Which two hemat diseases are treated with desmopressin?
Hemophilia A, vW disease (affiliated with FVIII)
Causes of DIC?
Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion, Rattlesnake venom , AML
Lab values of DIC?
Inc fibrin degradation, dec fibrinogen, Dec factors V and VIII
How does Antithrombin deficiency affect PT, PTT, and thrombin time? It can affect which of those values following an admin of which drug?
No direct effect on PT, PTT, or thrombin time. However, it can decrease the level of PTT increase following Heparin admin. (PTT is normally used to follow Heparin admin)
Nephrotic syndrome is associated with which two coagulation disorders?
DIC and Antithrombin deficiency
What type of mutation in FV Leiden?
DNA point mutation, G –> Arg506Gln
The mutation is FV Leiden def stops its degradation by what protein?
Prot C
Most common cause of hypercoagulability in Caucasians?
F V Leiden
What issue specific to women must one be concerned about in terms of FV Leiden def?
1) Inc risk of PE with OCP
2) Recurrent pregnancy loss
Prot C and S def will lead inability to inhibit which factors?
Factor Va and VIIIa
Be careful with prescribing which anti-coag when it comes to Prot C or S def?
Warfarin - inc risk of skin necrosis
location of Gene mutation of Prothrombin?
Mutation in 3’ UTR
Risk associated with blood transfusion?
transfusion reactions, Fe overload (could lead to 2ndary hemochromatosis) hypocalcemia, hyperkalemia
Viruses associated with Hodgkins and Non Hodgkins lymphoma?
EBV and HIV
Which type of lymphoma is assoc with B symptoms? What are those symptoms?
Hodgkin’s, low grade fever, night sweats, weight loss
CD # of Reed-Sternberg cells?
CD15, CD30
What are the two genes affected in Burkitts? (not the translocation itself, but the products of them)
Chr 8 - c -myc
Chr 14 - Ig heavy chain
Tingible body macrophages associated with?
Burkitts
What type of lesion is seen in the sporadic form of Burkitts?
Pelvis and abdomen lesions
Most common type of Non Hodgkin’s in adults?
Diffuse large B cell lymphoma
Why can blood transfusions lead to tingling sensations and paresthesias?
hypocalcemia
Gene products of the genes translocated in Mantle cell lymphoma?
Chr 11 - cyclin D1 (G1–> S)
Chr 14 - Ig heavy chain
What are the gene products of the gene translocation of Follicular lymphoma?
Chr 14 - Ig heavy chain
Chr 18 - bcl2
Which lymphoma presents with intermittent pain of lymph nodes?
Follicular lymphoma
What are the cell types in grades 1-3 of Follicular lymphoma?
Grade 1 - small cleaved cells
Grade 2 - Mixed
Grade 3 - large cells
Name an AIDS defining lymphoma?
Primary Central Nervous System Lymphoma
Presentation of PCNSL? Assoc with which virus?
Confusion, memory loss, seizures - assoc with EBV
3 diseases with mass lesions in CNS?
Toxoplasmosis, PCNL, GBM
Which lymphoma is associated with cutaneous lesion and tremor?
ATCL (Adult T cell ), tremor due to hypercalcemia, also associated with lytic bone lesions
Which condition is associated with lymphocytes with cerebriform nuclei? What CD #?
Mycosis Fungoides, CD 4+
Ig associated with M spike in Mult myeloma and in Waldenstrom macroglobulinemia?
MM - IgG, IgA
WM - IgM
What does the M in M spike mean?
M is not for IgM, but for Monoclonal - one clonal type of Ig
What type of amyloidosis seen in MM?
Primary (AL)
What are Bence Jones proteins?
Ig light chains
Name two signs of MM you’d see in a blood smear?
Stacked RBC (rouleaux) and plasma cells with “clock face” chromatin
The intracytoplasmic inculsions of plasma cells in MM contain what?
Ig
Clinical diff between MGUS and MM?
There are no Crab findings -
C - HyperCalcemia
R - Renal issues (due to BJ prot blocking tubules)
A - Amyloid, Anemia (plasma cells taking BM)
B - Bone lytic lesions, Back pain, Bence- Jones
What hematological issue has visual defect, bleeds, strokes, Raynauds?
Waldenstrom Macroglobulinemia - Sx from hyperviscosity
Neutrophils with bilobed nuclei seen after chemo?
Pseudo Pelger Huet anomaly
Causes of MDS?
de novo mutations, environmental (radiation, benzene, chemo)
MDS can lead to what type of leukemia?
AML
What is the Warburg effect, esp in relation to Burkitt’s
Cmyc - nucl phosphated prot –> transcription of activator that controls cell proliferation, differentiation, and apoptosis –> leads to upreg of anaerobic gycolysis = Warburg Effect
Warburg Effect - is the observation that most cancer cells predominantly produce energy by a high rate of glycolysis followed by lactic acid fermentation in the cytosol, rather than by a comparatively low rate of glycolysis followed by oxidation of pyruvate in mitochondria as in most normal cells.
Desmopressin also used in what neuro issue?
Enuresis
What disease looks very similar clinically to FV Leiden def?
Anti phospholipid syndrome
Deferoxamine is also used in what chemo drug tox?
Doxorubicin
MDS is most common acquired cause of heme issues?
Sideroblastic anemia
