6-03 Heme/onc

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fas

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cd95; cell surface receptor that triggers apoptosis after binding to fasL -- essential in eliminating activated lymphycotes (limiting immune response)

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perforin

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produced by CTLs -- drills holes thru target cells, followed by injection of cytotoxic proteins like granzyme b

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p53

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"guardian of the genome": blocks cell cycle at G1 after DNA damage until repaired, else activates apoptosis

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apoptosis vs necrosis

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apoptosis: cells shrink, organelles densely packed, cytoplasmic blebs, PERIPHERAL AGGREGATION OF CHROMATIN; necrosis: pale chromatin (karyolysis), irregular clumps, shrinking nucleus (pyknotic body), inflammation

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follicular lymphoma

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a type of small cleaved cell (b cell) lymphomas, t(14;18): b-cl2+IgH

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t{11;14}

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bcl-1+igH; a/w lymphocytic lymphomas, myelomas, some mantle-cell lymphoma

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t{8;14)

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Burkitt's lymphoma: c-myc+IgH

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tumor with ganglion cells and rosettes

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neuroblastoma (amplification of n-myc --> poor prognosis)

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c-myc vs n-myc

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c-myc in follicular lymphoma [t(14;18)]; n-myc in neuroblastoma

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salicylate vs acetaminophen tox presentation

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salicylate causes metabolic acidosis and respiratory alkalosis (RENAL problems); acetaminophen causes nausea, vomiting, abdominal pain, hepatic failure (GI problems)

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CO tox presentation

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hypoxia, cherry red coloration of lips / mucous membranes (CO-bound hemoglobin binds O2 very tightly, left-shift of hemoglobin curve)

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lead vs mercury posioning

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lead: anemia, neuropathy, GI problems; mercury: CNS atrophy, gingivitis, gastritis, RENAL tubular changes

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reason for increased gastric adenocarcinoma risk in Japanese

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diet: polycyclic hydrocarbons in smoked fish

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schistosomiasis

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liver/bladder/intestines injury (haematobium --> SCC of bladder;

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ITP vs TTP/MAHA

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ITP has no fragmented red cells (schistocytes) because it's immune mediated

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platelet threshold for generalized bleeding

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20000 platelets

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tingible body macrophages

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found in germinal center of lymph nodes, seen in benign reactive lymphadenitis (macrophages containing debris from apoptotic b cells)

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benign vs malignant lymphadenopathy

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benign is usually tender (!?)

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causes of decreased ESR

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cells settle slower: sickle cell anemia (altered cell shape), polycythemia (too many cells), CHF

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DIC associated with which leukemia

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AML type M3 (promyelocytic). t(15;17); auer rods contain tissue factor --> release --> clotting

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desmoplasia

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excess fibrous tissue formation in stroam of tumor

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erythroblastosis fetalis

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newborn, Rh or ABO incompatibility (mom's Abs attack fetal RBCs) --> weakly positive coombs dest, microspherocytosis

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Ames test

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measures damage to DNA -- correlates well with carcinogenicity; cheap, used for screening

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Watson-Schwartz test

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detects porphobilinogen in urine, used in dx of porphyria

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Widal test

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used to dx typhoid fever

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Woellner enzyme test

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monospot test

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porphyria steps

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succinyl CoA + glycine --> dALA --> prophobilinogen --> uroporphyrinogen III --> protoporphyrin --> heme

Hint 27

None

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rate limting step of heme synthesis

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succinyl CoA + glycine --------dALAsynthetase-----------> dALA

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fanconi anemia

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congenital aplastic anemia --> pancytopenia, hypoplastic bone marrow; a/w short stature, microcephaly, hypogenitalism, strabismus

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feverfew

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used to tx migraines (feverfew the band GIVES irun a migrane)

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causes of nystagmus

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PCP (not amphetamines), phenytoin

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None

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2 main causes of MAHA

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DIC and prosthetic valve

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primary amyloidosis

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Ig light chains (AL protein = amyloid light chain) deposition; deposits in tissues of mesodermal origin (heart, muscle, tongue, kidney)

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secondary amyloidosis

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AA protein (formed from SAA, serum amyloid-asociated protein); involve parenchymal organs: KIDNEY, liver, adrenals, pancreas, lymph nodes, spleen; usu 2' to chronic inflam disease like RA, TB, osteomyelits, syphilis, leprosy, etc.

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anisocytosis

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varying sizes

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poikilocytosis

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varying shapes

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basophil vs mast cell

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basophils in blood, mast cells in tissue; both involved in allergic (type I hypersensitivity) reaction -- release histamine and heparin

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causes of eosinophilia

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NAACP (Neoplasm, Asthma, Allergy, Collagen vascular diseases - eg Churg-Strauss, Parasites)

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churg-strauss syndrome

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type of PAN, aka 'allergic granulomatosis'; small/medium vessel autoimmune vasculitis --> necrosis; a/w eosinophils, granulomas, and pANCA

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definition of serum

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plasma - clotting factors

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dense granules

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ADP, Ca++, vasoactive amines

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alpha granules

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fibrinogen, vWF

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kallikrein

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serine protease formed from prekallikrein by factor XIIa; used to convert HMWK (kininogen) --> bradykinin and plasminogen --> plasmin

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tumor grade vs stage

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grade = degree of cellular differentiation; stage = degree of spread (TNM system: Tumor size, Node involvement, Metastases); stage generally has greater prognostic value

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Brain mets

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50% of brain tumors are mets. "Laura Bush's Skin Kills Glia" (Lung, Breat, Skin-melanoma, Kidney, GI)

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None

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Liver mets

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"Cancer Sometimes Penetrates Benign Liver" (Colon > Stomach > Pancreas > Breast > Lung)

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Bone mets

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"P.T. Barnum Loves Kids:" Prostate, Thyroid, Testes, Breast (Both lytic and Blastic), Lung (Lytic), Kidney; Breast and Prostate are most common; Mets >> 1' bone tumors

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Cancer Epi, Men

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Incidence: Prostate > Lung > Colon/Rectum; Mortality: Lung > Prostate

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Cancer Epi, Women

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Incidence: Breast > Lung > Colon/rectum; Mortality: Lung > breast (replace male Prostate with Breast)

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Acanthocyte

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spur cell (liver damage, eg abetalipoproteinemia)

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Burr Cell

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Renal disease (TTP/HUS)

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distribution of alpha vs beta thal

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alpha in africa/asia; beta in mediterranean

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HbBarts

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HbBarts = gamma4, occurs in alpha thal major (4 missing alpha genes --> hydrops fetalis) -- "Bart's just a KID (hydrops fetalis) who plays with his SLINGSHOT (gamma globins)"

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HbH

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beta4 tetramers, seen in alpha thal minor (3 genes missing)

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beta thal hemoglobins

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major: HbF (alpha2gamma2 -- fetal hemoglobin); minor: HbA2 (alpha2delta2) + HbF

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sickle-cell thal

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HbS/B-thal heterozygote: mild to moderate disease

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drug causes of aplastic anemia

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benzene, chloramphenicol, NSAIDs, alkylating agents, anti-metabolites

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non-drug causes of aplastic anemia

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radiation, VIRAL (B19, EBV, HIV), Fanconi's anemia, idiopathic, post-ACUTE HEPATITIS

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fanconi anemia

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AR, defect in DNA repair --> marrow hypofunction, along with hypoplasia of kidney and spleen.

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crew cut' on xrays

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sickle cell or thal -- indicates extramedullary hematopoeisis

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complications of sickle cell

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1) aplastic crisis after parvovirus B19; 2) osteomyeltis from Salmonella; 3) autosplenectomy; 4) painful (vaso-occlusive) crisis in deoxygenated/cold/dehydrated environments

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cold vs warm agglutinin HA

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cold: IgM, ACUTE, triggered by cold, a/w recovery from mycoplasma pneumo / infections mononucleosis infection; warm: IgG, CHRONIC, a/w SLE, CLL, drugs (eg alpha-methyldopa); both cause spherocytosis, coombs positive

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hereditary spherocytosis vs autoimmune anemia

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both have spherocytosis; HS is coombs negative and can be detected by osmotic fragility test; HS due to ankyrin/spectrin defect

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PNH

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paroxysmal nocturnal hemoglobinuria -- defect in pig1 gene --> defective GPI anchor for proteins that inhibit complement-mediated destruction ==> hemolysis by complement, increased urine hemosiderin

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causes of DIC

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MCC: obstetric complications (toxemia, amniotic fluid embolus, abruptio placentiae); others: G- sepsis, transfusion, trauma, malignancy

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ITP

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Idiopathic/Immune Thrombocytopenic Purpura: self-limtiing in kids, chronic in adults; anti-platelet IgGs cause platelet destruction and increased production (increased megakaryocytes)

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TTP

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Thrombotic Thrombocytopenic Purpura: ADAMTS13 mutation (vWF metalloprotease deficiency) --> increased vWF --> clumping --> MAHA, depletion of platelets; a/w neuro abnormalities and renal dysfunction (like HUS)

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platelet abnormalities

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1) ITP; 2) TTP; 3) DIC; 4) Aplastic anemia; 5) Drugs (immunosuppressive); ==> microhemorrhage, increased BT

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Coag factor defects

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1) Hemophilia A (VIII); 2) Hemophilia B (IX); 3) vWD (defective platelet adhesion, \/ VIII); ==> MACROhemorrhage (hemarthroses, easy bruising, /\ PT and/or PTT)

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Henoch-Schonlein Purpura

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post-infectious, IgA hypersensitivity vasculitis, hemorrahagic urticaria; GI/renal involvement (IgA nephropathy)

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Osler-Weber-Rendu syndrome

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aka Hereditary Hemorrhagic Telangiectasia -- AD, localized venous malformations --> hemorrhage

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bernard-soulier

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defect of GP Ib

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glanzmann's thrombasthenia

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defect of GP Iib/IIIa

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Hodgkin's lymphoma

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Reed Sternberg cells (CD15+ CD30+), localized axial lymphadenopathy, extranodular nodes rare, constitutional sx seen in type B, EBV assoc, bimodal age distribution (young and old), more common in men; good progress: inc lymphocytes, dec RS cells

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Non-Hodgkin's lymphoma

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multiple lymphnodes, peripheral, noncontiguous spread; usus B-Cell, a/w HIV/immunodeficiency; fewer constitutional sx than HL, peak age 20-40 (inverse of HL)

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Hodgkin's lymphoma epi

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bimodal age distribution (young and old); more common in men except for nodular sclerosing (most common type -- women > men, young adults)

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hodgkin's lymphoma Nodular Sclerosing subtype

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NODULAR SCLEROSING: most common, f>m, 20s, excellent prognosis, collagen banding, no a/w EBV, *lacunar* variant of RS cell;

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hodgkin's lymphoma, Non-NodularSclerosing subtypes

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LYMPHOCYTE PREDOMINANT: young males, excellent prognosis, lots of lymphocytes; LYMPHOCYTE DEPLETED (rare): older males, poor prognosis; MIXED CELLULARITY (more common than both): in between in # lymphocytes and in prognosis

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ALL

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KIDS, most responsive to Tx, can spread to CNS / testes

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AML

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adults, Auer rods; M3 subtype = APML, t(15;17), responsive to all-trans retinoic acid; a/w DIC (release of auer rods with treatment)

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CLL

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older adults, hepatosplenomegaly, indolent, SMUDGE CELLS, v.similar to SLL (small lymphocytic lymphoma)

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CML

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Ph chromosome t(9;22); splenomegaly, elevated PMNs; can progress to AML (BLAST CRISIS);

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CML vs leukemoid reaction

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elevated leukocyte alk phosphatase in leukemoid reaction, very low in CML

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t(11;22)

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ewing's sarcoma

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t(8;14)

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Burkitt's lymphoma: c-myc+IgH

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t(11;14)

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mantle cell lymphoma: bcl-1 / Cyclin D1

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t(14;18)

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follicular lymphoma: bcl-2

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t(15;17)

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M3 type of AML (responsive to all-trans retinoic acid)

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Multiple myeloma vs Waldenstrom's

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both have M-spike on SPEP, rouleux formations, bence jones proteins; waldenstrom's has hyperviscosity, NO lytic bone lesions

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abciximab

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mab against GP Iib/IIIa receptors on activated platelets --> prevents aggregation

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consequences of warfarin-induced inhibition of protein C

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skin necrosis (seen in first week of warfarin tx)

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steps of tumor invasion

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1) detachment (downregulation of e-cadherins); 2) adherence to basement membranes (increased laminin, other adhesion molec); 3) invasion of BM (proteolytic secretions, including metalloproteinases, cathepsin D)

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what binds integrins

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collagen, fibronectin, and laminin

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geographic types of burkitt's lymphoma

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african: endemic, jaw involvement, a/w EBV; american: visceral organ involvement, sporadic

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burkitt's vs follicular

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burkitt's: kids, t(8;14), jaw involvement (african type); follicular: adults, t(14;18), generalized lymphadenopathy

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dangerous vitamin deficiency in preterm infants

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Vit K: hepatic immaturity, gut flora not well established, low vit k content of breast milk (also no vit D)

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role or Rb

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regulates G1 --> S transition; phosphorylation --> E2F --> goes through checkpoint

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regulation of iron

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enterocytes: absorbed iron either binds transferrin in blood or ferratin in enterocyte; when dietary iron is high, intestinal cells decrease the amt abosrbed

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diff in labs of Factor V Leiden and Antiphospholipid Ab syndrome

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antiphospholipid antibody syndrome has elevated PTT (paradoxically, since it's a thrombotic disorder) -- in vitro artifact

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heme effects of folic acid deficiency

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homocysteinuria (folic acid required for homocysteinuria --> methionine) ==> prothrombotic state

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histo of follicular lymphoma

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at low mag, aggregates of packed follicles that obscure normal lymph node architecture

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lipofuscin vs hemosiderin

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hemosiderin is from iron excess, lipofuscin is from "wear and tear"; both look yellow-brown

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ALL vs AML

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ALL seen in KIDS

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anemia a/w placenta abruptiae

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DIC

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tests for DIC

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platelet count (low), fibrinogen levels (low), fibrin degradation products (high)

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ALL L2

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ALL-T

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ALL L3

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ALL-B ("3 looks like a B")

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AML M2 and M3

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APML

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AML M7

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Megakaryocytic differentiation

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AML M6

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Erythroid differentiation

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AML M5

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monoblastic / monocytic

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AML M4

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myeloid AND monocytic

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AML M1 --> M7

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increasing differentiation

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AML M1

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undifferentiated

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mechanism of thrombolytics (streptokinase, urokinase, tPA)

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bind and cleave plasminogen --> plasmin --> fibrin cleavage

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type of anemia in renal failure

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normochromatic, normocytic (decreased epo)

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main component of eosinophil granules

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major basic protein --> destruction of parasites

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target cells a/w

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liver disease, thalassemia, and HbC

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WBC diff of pts with CML

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increased PMNs and metamyelocytes, decreased eosinophils, basophils, and a few blasts

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TTP vs HUS

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no CNS effects in HUS

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DIC vs TTP

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DIC has elevated PT and PTT, decreased fibrinogen, and elevated fibrinogen split products; also, DIC doesn't have renal involvement?

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lab effects of tPA administration

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increased PT and PTT, no change in platelets

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pathogenesis of EBV mono

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EBV invades B-cells using CD-21 receptor, causes hyperproliferation --> lymphadenopathy and splenomegaly; in peripheral smear, would see elvated CD8+ CTLs