Hematolgy + Onkology Flashcards
What is Aculte lmphoblastic leukemia/ lymphoma?
more in children, T-cell ALL can present as mesdiastinal mass, associated with Down syndrome, tdt, CD10+
t(12;21)
increased lymphoblasts
all-trans retinoic acid, Vit A
Rbc inclusions
what is seen in sideroblastic anemia eg. lead poisoning?
basophilic stippling, they are ribosomal precipitates
what is seen in g6pd?
Heinz bodies due to oxidative stress which cause hb denaturing and precipitates
what can be seen in hypoplenia and aplenia?
basophilc nuclear remnants found in rbcs
howel-jolly bodies are removed from splenic macrophages
what can be found in megalobastic anemia?
impaired dna, hypersegmented neutrophils
What are the causes of folate deficiency?
malnutrition: alcohol, malabsorption, drugs: metotrxate
Multiple myeloma
What appears in MM?
think CRAB:
hyperCalcemia Renal involvement Anemia Bone lytic lesions/ Back pain Multiple Myeloma: Monoclonal M-protein
Monoclonal plasma cells: fried egg appearance, IgG and IgA
Clock face
Rouleaux formation
dipstick: is negative only Albumen and Bence Jones proteins.
Burkitt lymphoma translocation
Burk-8 t(8;14) c-myc and BCL2 genes, stary sky
Mantel cell lymphoma
t (11;14) cycline D1 activation
Marginal zone lymphoma
t(11:18), associated with chron. infections eg. Sjörgen, MALT Lymphoma, CD5 very aggressive
Follicular lymphoma
t(14;18) heavy chain Ig 14 BCL2 inhibits apoptosis, present with painless waxing and waning
APL
t(15;17) M3 type of AML; respondsto all-trans retinoic acid
CML
t(9;22) Philadelphia chromosme BCR-ABL hybrid
sideroblastic anemia
gen. x-linkeddefect of ALA synthase gene, aquired (myolodysblastic syndrome) and revesible (alcohol, lead, vit. b6 defiency, copper deficiency, drugs like izoniazid, linozolid
increased iron and ferritn
lead poisining
Ferrochelate and ALA dehydratsae def., accumulation of protoporphyrin, ALA leads to mirocytic aneamia and
ringed sideroblasts in bone marrow
Acute intermittent porhyria
Porphobilinogen deaminase, previously known as uroporphyrinogen 1, auto. domi., accu. of porphorbilinogen, ALA. Symptoms 5Ps: Painful abdome, Portwine couloured urine, polyneropathy, psychlogical disturbances, Precipitation by drugs
Treatment and glucose
porphyria cutanea tarda
Uropophyrinogen decaboxylase, accu. of Uroporphyrin (tea colored urine).
Blistering due to sun
Treatment: phlebotomy, antimalrials
Sideroblastic anemia (X-linked)
aminolevulinic acid synthase
Lead poisoning
Aminolevulonic acid dehydratase
Hodgkin lymphoma
Reed-Sternberg cell (owl eyes) distinc giant cells
CD 15, CD 30
2 owl eyesx 15= 30
NON Hdgen lymphoma
B-cells, associated with EBV, HIV
Diffuse b-cell lymphoma
BCL2 and BCL6, most common lymphoma
prim. central nervous system lymphoma
AIDS
Adult T-cell lymphoma
IV drug abuse, T-cell for Tokyo
CLL
60> CLL= crushed little lymphocytes= smudge cell, most common CD20, CD23, CD5+ B-cell neoplasm
Hairy cell leukemia
adult males, mature b-cells. cells with hairlike projection. Marrow fibrosis= dry tap. Stains Trap: tartate-resitant acid phosphate
TRAP-ped in Hairy situation
treament: clabdribine, pentostatin
AML
65>, Auer rods, myoloperoxidase, cytoplasmic inclusions
CML
45> 9:22 Philadelphia BCR-ABL, low LAP, responds to thyrosine kinese inhibitors
Heparin
activation of antithrombin which activates IIa and factor Xa, follow PTT
direct thrombin inhitors are? mech of action?
BAD, Bivalirudin, Argatroban, Dabigatran
inhitis thrombin
Thrombolytics
(tPA) Alteplase, streptokinase
direct and indtrect aid conversion of plasminogen to plasmin, increase in PT and PTT
ADP rectoptor inhibitores,
irri. blocks ADP receptor and prevent glycoprotein IIb/llIa on platelets
Clopidogrel, ticagrelor
glycoprotein IIb/IIIa inhibitor
Abximab (monoclonal ab Fab fragment), binds glycoprotein IIb/IIIa on the activated platelets, preventing aggregation
thrombotic thrombocytopenic purpura
TTP:inhibition or deficiency of ADAMT13 (a vWF metalloprotease) increased platelet adhesion and aggregation——> microthrombi
Presentation: triad -thrombocytopinia, microangiopathic hemolytic aneamia, acute kidney injury
+fever and neurologic symptoms
Labs: platelet count is low, schitocytes, normal PT/PTT
hemolytic uremic syndrome
HUS: commonly caused by shiga-like toxin from EHEC. atypical is caused by caused by complement gene mutation
Presentation: triad -thrombocytopinia, microangiopathic hemolytic aneamia, acute kidney injury
+bloody diarrhea
Labs: platelet count is low, schitocytes, normal PT/PTT
DIC
Hydroxyurea mech?
inhibits ribonucleotide reductase
Bleomycin mech?
DNA breakage, SE: pulmonary fribosis, USE: Hodgkin lymphoma
Dactimycin, doxyrubicin mech?
DNA intercalators
Etoposide mech?
topomerase II
Irotecan mech?
topmerase I
Paclitaxil mech? and other drugs
hyperstabilized microtubule, inhibit microtubles Vinblastin (blasts the bone marrow), Vincristine (crisps the nerves)
Cisplatin mech?
platinum agent, cell cycle independent, nephrotoxic
Cladribine
Purine analogon, inhibition of DNA polymerase, USE: Hairy cell leukemia
Cystarabine
Pyrimidine analog, inhibits DNA polymerase, USE: leukemias
Busulfan
Cross-linked DNA, USE: before bone marrow tranplant
Cyclophosphamide
Cross-linked DNA at guanine, USE: leukemias
BeVacizumab
M. ab aginast VEGF, inhibits angiogenisis. BV for blood vessels. USE: Solid Tumor eg colorectal
Erlotinib -inib
EGFR tyrosine kinase inhib. USE: NON-small cell cancer
Myelofibrosis
Obliteration of bone marrow with fibrosis, teardrop RBCs, bonemarrow is crying
Transferrin
Ferritin
Transferrin: tranport
Ferritin: storage
Target cells
HALT, said the hunter to his target HbC, Asplenia, Liver disease, Thalassemia
Anemia in chronic disease
IL6 increased, hepcidin increased
Aplastic anemia
failure or destruction of hematopoeitic stem cells eg Radiation, Drugs . Dry bone tap (Fatt in the bone marrow)
reticolos low, EPO increased
Autoimmune hemolytic anemia
Warm IgG- chronic seen in SLE and CLL
Cold IGM- acute seen in CLL, Mycoplasma and Mononucl. direct and indirect combs
Microangiopathic anemia
HUS etc
Macroangiopatic anemia
Heart valve and aortic stenosis
Infectios anemia
Babesia and other infec. destr. of Rbcs
antithrobin deficiency
inherited defi. no effect on PT PTT
Factor V Leiden
mutation of factor V (guanin—>adenin DNA point mutation, degradation of protein C
inher. hypercuagulability, DVT, pregnancy loss
Protein C or S defiency
decreased ability to inactvated factors Va and VIIIa, skin necrosis for warfarin. Together C= Cancels and S=Stops coagulation
immune throbocytopenia
destruc. of platelets in spleen. anti gbIIb/IIIa ab eg autoim. SLE, viral HIV and malignant CLL.
increased megakariocytes
Gaucher disease
beta-glucocerebrosidase, histocyses wrinkeled toilet paper
PT, PTT, INR, Bleeding time
PT: 11-13,5s PTT:25-35 INR:1,1 (2-3 terapeudic range)
