Term
|
Definition
Source of energy is glucose (90% glycoloysis, 10% HMP shunt)
Contains HCO3/Chloride anti porter which allows RBCs to sequester HCO3 |
|
|
Term
|
Definition
Derived from Megakaryocytes
Dense granules (ADP, calcium)
Alpha granules (vWF, fibrinogen)
Platelets sequestered in spleen |
|
|
Term
|
Definition
Neutrophils: 54-62%
Lymphocytes: 25-33%
Monocytes: 3-7%
Eosinophils: 1-3%
Basophils: 0-0.75% |
|
|
Term
|
Definition
Phagocytic, multilobed
Small Granules contain: AlkPhos, collagenase, lysozyme, and lactoferrin
Azuophilic granules: acid phsophatase, peroxidase and B-glucuronidase
*hypersegmented ploys are seen in Vit B12 and folate def
Inc band cells indicates myeloid proliferation |
|
|
Term
|
Definition
Differentiate into macrophages
*Large kidney shaped nucleus, Frosted glass cytoplasm
Joints- A cells, Liver- Kupffer cells, Kidney- mesangial cells |
|
|
Term
|
Definition
*CD14, Long life in tissues
Activated by g-interferon
Function as APC via MHC 2
Secretes IL-1, IL-16, TNF-a
*giant cells can make vitamin D |
|
|
Term
|
Definition
Major Basic Protein, for protection form helminths
Highly phagocytic for Ag/Ab complexes
Prod: Histamine, Arylsulfatase (limit rxn following degran) |
|
|
Term
|
Definition
| Neoplastic, asthma, allergic processes, collagen vascular diseases, parasaites |
|
|
Term
|
Definition
Mediates allergic rxn
Ganules: heparin, histamine, leukotrienes |
|
|
Term
|
Definition
Mediate allergic rxn in local tissues
Similar to basophils in structure and function
Can bind Fc portion of IgE to membrane
IgE causes degranulation, upon cross linking
Releases Histamine, Heparin and eosinophil chemotactic factors
*type 1 HS Rxn
Tx: Cromalyn Sodium prevents mast cell degranulation |
|
|
Term
|
Definition
Highly phagocytic APC
Expresses MHC 2 and Fc receptor on surface
Called Langerhans cells in the skin
CD 40, 80, 86 |
|
|
Term
|
Definition
Migrates from BM to peripheral lymphoid tissue
When Ag is encountered, B cells differentiate into plasma cells that produce Ab and memory cells
*Can function as APC via MHC 2
CD 19, 20, 21; IgM and IgD on surface |
|
|
Term
|
Definition
Produces large amount of Ab
*Clock face chromatin, abundant RER, well dev Golgi |
|
|
Term
|
Definition
Cytotoxic- CD8, rec MHC 1
Helper- CD4, rec MHC 2
Regulatory- CD 28 |
|
|
Term
|
Definition
A- A ag on surface, Anti B in blood
AB- universal recipient of RBCs, universal donor platelets
O- universal donor RBCs, universal recipient plasma
*Anti A or B Abs are IgM, do not cross placenta
Tx: Rho (d) Ig to prevent sensitization to Rh |
|
|
Term
|
Definition
| 7 activated by thromboplastin (TF) > 10 |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Oxidized Vit K > reduced (via epoxide reductase) > mature coag factors
Inhibited by Warfarin
vWF carries/protects F8 |
|
|
Term
|
Definition
Protein C > activated protein C (thrombomodulin) > cleaves and inactivates 5, 8 (with protein S)
Antithrombin inhibits active forms of 2, 7, 9, 10, 11, 12
Heparin activates antithrombin
Factor v leiden resists inhibition by protein C |
|
|
Term
|
Definition
1. vWF binds exposed collagen upon damage
2. Platelets bind vWF (gp1), release ADP and Ca (for coag), ADP helps platelets adhere to endothelium
3. ADP binding expresses Gp2 on platelet surface
4. Fibrinogen binds Gp2 and links platelets |
|
|
Term
|
Definition
Spur cell
Liver disease, abetalipoproteinemia
*cholesterol dysregulation |
|
|
Term
|
Definition
| Thalassemias, anemia of chronic dz, Lead poisoning |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Megaloblastic anemia
Marrow failure |
|
|
Term
|
Definition
Sideroblastic anemia
Excess iron in mitochondria |
|
|
Term
|
Definition
Helmet cells
DIC, TTP, HUS
Anything that causes traumatic hemolysis |
|
|
Term
|
Definition
Hereditary spherocytosis
Autoimmune hemolysis |
|
|
Term
|
Definition
Bone marrow infiltration
Myelofibrosis |
|
|
Term
|
Definition
| HbC disease, Asplenia, liver dz, Thalassemia |
|
|
Term
|
Definition
Oxidation of Hgb sulfhydral group
Denatured Hgb precipitation and damage to RBC membrane
G6PD deficiency, alpha thalassemia |
|
|
Term
|
Definition
Basophilic nuclear remnant
Hyposplenia or asplenia
*mothball ingestion (napthalene) |
|
|
Term
|
Definition
| Iron deficiency, alpha thalassemia, Beta thalassemia, lead poisoning, sideroblastic anemia |
|
|
Term
|
Definition
Dec iron due to chronic bleeding, malnutrition, or pregnancy
May manifest as Plummer Vinson Syndrome (Fe def anemia, glossitis, esophageal webs) |
|
|
Term
|
Definition
Defect in a-globin synthesis
cis deletion prevalent in Asian populations
trans deletion prevelent in AA populations
4 gene deletion- forms Hg Barts, not comparable with life
3 gene deletion- HbH dz, excess B globin forms tetramers
2 gene deletion- No clinical significance |
|
|
Term
|
Definition
Point mutations in splice sites and promoter sequences
Prevalent in Mediterranean people
Minor- Inc HbA2, asymptomatic
Major- Severe anemia, marrow expansion (crew cut), chipmunk facies, inc HbF (a2g2) |
|
|
Term
|
Definition
Lead inhibits ferrochelatase and ALA dehydratase
*Leads to dec heme synthesis
Also inhibits rRNA degredation, causing RBCs to retain aggregates of rRNA
Lead lines on gingiva and metaphases of long bones
Encephalopathy and erythrocyte basophilic stippling
Wrist and foot drop
*Dimercaprol and EDTA, Succimer for adults |
|
|
Term
|
Definition
Defect in heme synthesis
X linked defect in d-ALA synthase gene
Iron laden mitochondria
Inc iron, normal TIBC, inc ferritin
Tx: Pyridoxine (B6 cofactor for ALA synthase) |
|
|
Term
|
Definition
| Folate def, B12 def, orotic aciduria |
|
|
Term
|
Definition
Alcoholic malnutrition, methotrexate, trimethorpim, pheyntoin, pregnancy
Hypersegmented neutrophils, glossitis,
*inc homocysteine, but normal methylamalonic acid |
|
|
Term
|
Definition
Insufficient intake (vegans), malabsorption, pernicious anemia, Diphyllobothrium latum (fish tapeworm), PPIs
Same things as folate def, +neurologic symptoms
Inc homosyteine, INC methylamalonic acid
Subacute combined degeneration: peripheral neuropathy, post columns, lateral corticospinal, dementia |
|
|
Term
|
Definition
Genetic mutation in enzyme that synthesizes uridine from orotic acid
*megaloblastic anemia cannot be cured by B12 or folate
Orortic acid in urine
Tx: urudine monophosphate to bypass enzyme |
|
|
Term
| Non megaloblastic macrocytic anemias |
|
Definition
Macrocytic anemia in which DNA synthesis is unimpaired
Liver disease, alcoholism, 5-FU, AZT, hydroxyurea |
|
|
Term
| Hemolytic Normocytic, normochromic anemias |
|
Definition
Intravascular hemolysis- Dec haptoglobin, Inc LDH
PNH, mechanical destruction
Extravascular hemolysis- macrophage in spleen clears RBCs
Inc LDH, plus Inc UCB
Hereditary Spherocytosis |
|
|
Term
| Non hemolytic normocytic, normochromic, anemias |
|
Definition
| Anemia of chronic dz, aplastic anemia, chronic kidney disease |
|
|
Term
|
Definition
Inflammation, inc hepcidin, binds ferroportin and macrophages, inhibiting iron transport (sequestration)
dec iron, inc ferritin, dec TIBC |
|
|
Term
|
Definition
Failure or destruction of the myeloid stem cells due to:
Radiation or benzene, chloramphenicol, alkylating agents, antimetabolites
Viruses- Parvovirus B19, EBV, HIV, HCV
Fanconis anemia- DNA repair defect
*normal cell morphology but hypocellular bone marrow |
|
|
Term
|
Definition
| Dec EPO > dec hematopoiesis |
|
|
Term
|
Definition
Defect in spectrin and ankyrin in PM
Small and round erythrocytes with no central pallor
Inc MCHC, Inc RDW
Splenomegaly, aplastic crisis
Pos osmotic fragility test
Tx: splenectomy |
|
|
Term
|
Definition
X linked defect in G6PD
Dec glutathione, Inc RBC susceptibility to odidant stress
Primarily destroyed extravascularly
Back pain, hemoglobinuria a few days later
Heinz bodies and bite cells |
|
|
Term
| Pyruvate Kinase deficiency |
|
Definition
AR, defect in pyruvate kinase
Dec ATP, rigid RBCs
Hemolytic anemia in a newborn |
|
|
Term
|
Definition
Gutamic acid to Lysine mutation at residue 6 in B globin gene
Milder than HbSS patients |
|
|
Term
| Paroxysmal nocturnal hemoglobinuria |
|
Definition
Inc complement mediated RBC lysis
*impaired synthesis of DAF or GPI anchor that usually protect RBC from complement lysis
Triad- hemolytic anemia, pancytopenia, venous thrombosis
CD55/59
Tx: eculizumab |
|
|
Term
|
Definition
Glutamic acid for valine change in B globin gene
Low O2 or dehydration precipitates sicking
Newborns have HbF so asymptomatic
Crew cut appearance on XR due to erythropoiesis
Aplastic crisis, autosplenectomy
*salmonella osteomyelitis
Renal papillary necrosis and microhematuria
Tx: hydroxyurea to inc HbF |
|
|
Term
| Autoimmune hemolytic anemia |
|
Definition
*Coombs positive
Warm Agglutinin- IgG
- SLE, CLL, a-methyldopa
Cold agglutinin- IgM
- CLL, M pneumonia, Mono |
|
|
Term
|
Definition
RBCs damaged when passing through obstructed or narrowed vessel lumin.
DIC, TTP, HUS, SLE
Schistocytes seen in blood smear |
|
|
Term
|
Definition
Prosthetic heart valves and aortic stenosis may cause hemolytic anemia
Schistocytes on blood smear |
|
|
Term
| Infections causing anemia |
|
Definition
|
|
Term
|
Definition
Ferrochelatase and ALA dehydrates are affected
Potoporphyrin and d-ALA accumulate
Microcytic anemia, GI and kidney disease
Headache, memory loss, demyelination |
|
|
Term
| Acute intermittent Porphyria |
|
Definition
Porphobilinogen deaminase affected
Porphobilinogen, d-ALA, uroporphyrin build up
Painful abdomen, Port wine colored urine, polyneuropathy, psychological disturbances, precipitated by drugs
Tx: glucose and heme |
|
|
Term
|
Definition
Uroporphyrinogen decarboxylase accumulates
Uroporphyri (tea colored urine)
Blistering cutaneous photosensitivity |
|
|
Term
|
Definition
Hemarthroses
Both cause inc PTT |
|
|
Term
|
Definition
Dec PC, Inc BT
Defect in Gp1b > no platelet to vWF adhesion |
|
|
Term
| Glanzman's thrombasthenia |
|
Definition
Defect in Gp2b/3a > no platelet - platelet aggregation
blood smear shows no platelet clumping |
|
|
Term
|
Definition
Dec PC
Anti-Gp2b/3a antibodies
Splenic macrophages eat the complexed platelets-Abs
Inc megakaryocytes on labs |
|
|
Term
|
Definition
Dec PC
Def in ADAMTS13 (vWF metalloprotease)
Decreased degradation of vWF multimers
Inc platelet aggregation and thrombosis
Shistocytes and Inc LDH on labs
*fever, thrombocytopenia, and microangiopathic hemolytic anemia |
|
|
Term
|
Definition
inc BT, poss increase in PTT
Intrinsic pathway defect; vWF carries and protects factor 8
Autosomal dominant- most common bleeding disorder
Dx: ristercetin cofactor assay
Tx: DDAVP (desmopressin) which releases endothelium vWF |
|
|
Term
|
Definition
Inc BT, PT and PTT
Widespread activation of clotting leads to a deficiency of clotting factors
Sepsis, trauma, obstetric complications, acute pancreatitis, malignancy, nephrotic syndrome, transfusion
Inc D-dimer (fibrin split products) |
|
|
Term
|
Definition
| Factor V resistent to PC or PS degredation |
|
|
Term
| Prothrombin gene mutation |
|
Definition
Mutation in 3' untranslated region- Inc production of prothrombin
Inc plasma levels and venous clots |
|
|
Term
|
Definition
Inherited deficiency
*Inc PTT is blunted after heparin administration |
|
|
Term
| Protein C or S deficiency |
|
Definition
Dec ability to inactivate factors 5 and 8
*Inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin |
|
|
Term
|
Definition
Acute inflammatory response to infection
Inc WBCs with inc neutrophils and neutrophil precursors
Inc leukocyte Alk Phos
Contrast with CML (no Alk phos) |
|
|
Term
|
Definition
Single group of nodes, contiguous spread (stage is best pred)
*Reed-Sternberg Cells
Young adulthood and old age (more common in men)
50% associated with EBV
Low grade fever, night sweats, weight loss |
|
|
Term
|
Definition
Distinctive tumor giant cell seen in Hodgkins disease
"Owls eyes"
CD30, CD15 pos (B cell origin)
Nodular sclerosing most common (men and women equally)
Lymphocyte mixed or depleted forms have poor prognosis |
|
|
Term
|
Definition
Adolescents or young adults
*8;14 translocation of cmyc and heavy chain
Starry sky appearance
Assoc with EBV
Jaw lesion seen with endemic form in Africa |
|
|
Term
| Diffuse Large B cell lymphoma |
|
Definition
Older males usually
Most common adult NHL
May be mature T cell in origin |
|
|
Term
|
Definition
Older Males
11:14 translocation of cyclin D1 to heavy chain
Poor prognosis, CD5 |
|
|
Term
| "Dismantle the Follicle or Burn it" |
|
Definition
|
|
Term
|
Definition
Adults
14;18 translocation of heavy chain and bcl2
Difficult to cure, indolent course |
|
|
Term
|
Definition
Caused by HTLV-1
Adults present with cutaneous lesions; especially affects populations in japan, west africa and the caribbean
*aggressive |
|
|
Term
| Mycoses fungoides/Sezary syndrome |
|
Definition
Cutaneous patches/nodules, CD4
Indolent course |
|
|
Term
|
Definition
Fried egg cells
Produces large amounts of IgG or IgA
Assoc: amyloidosis, punched out lesions of bone, M spike, Bence jones protein, Rouleux formation
Normal plasma cells with clock face chromatin
Hypercalcemia, renal insufficiency, anemia, bone lesions |
|
|
Term
| Waldenstroms macroglobulinemia |
|
Definition
M spike, IgM
No lytic bone lesions, but similar to MM |
|
|
Term
|
Definition
Monoclonal expansion of plasma cells with M spike
Develop MM at a rate of 1-2% per year |
|
|
Term
|
Definition
Age < 15
Mediastinal mass
Markedly increased lymphoblasts "solid sheets"
TdT+, CALLA+
*most responsive to therapy
May spread to CNS and testes
12;21 translocation better prognosis |
|
|
Term
|
Definition
Age >60 yrs
Smudge cells in blood smear, AI hemolytic anemia
CLL has increased peripheral blood lymphocytosis or BM involvement
SLL- LN only |
|
|
Term
|
Definition
Mature B cell tumor in the elderly
Filamentous hair like projections
*TRAP positive (Hair in the trap)
Tx: Caldribine (adenosine analog) |
|
|
Term
|
Definition
Onset >65 yrs
*Auer rods
Markedly increased circulating myeloblasts
15;17 translocation
*M3 subtype responds to alltrans retinoic acid
DIC is a common presentation |
|
|
Term
|
Definition
30-60 yrs old
Philadelphia chromosome - 9;22 translocation (bcr-abl)
Inc neutrophils, metamyelocytes, basophils
Splenomegaly
May accelerate into AML or ALL
Very low leukocyte alk phos
Tx: imatinib (inhibitor of the tyrkinase bcd-abl) |
|
|
Term
|
Definition
| peroxidase positive cytoplasmic inclusions in granulocytes and myeloblasts |
|
|
Term
| Langerhans cell histiocytosis |
|
Definition
Proliferative disorders of dendritic cells from monocytic lineage
Lytic bone lesions and skin rash
Do not efficiently stimulate T cells with Ag
S-100 and CD1a
Birbeck granules, tennis rackets |
|
|
Term
|
Definition
| PCV, essential thrombocytosis, myelofibrosis |
|
|
Term
| Appropriate absolute polycythemia |
|
Definition
Inc RBC mass, dec O2 saturation
Lung dz, congenital heart dz, high altitude |
|
|
Term
| Inappropriate absolute polycythemia |
|
Definition
| RCC, wilms tumor, cyst, hepatocellular caricnoma, hydronephrosis, ectopic erythropoietin |
|
|
Term
|
Definition
Acitvation of antithrombin, dec thrombin and factor 10
Safe in pregnancy
T- HIT
Monitor PTT for effectiveness
LMW- enoxaparin and dalteparin act on factor 10, longer half life, not easily reversed |
|
|
Term
|
Definition
Direct thrombin inhibitor
Alternative to heparin for people with HIT |
|
|
Term
|
Definition
Development of IgG Ab against heparin bound to PF4
Thrombosis and thrombocytopenia |
|
|
Term
|
Definition
Interferes with g-carboxylation of vitamin K del factors
Blocks epoxide reductase
Metabolized by p450
For chronic coag and atrial fibrillation
Monior PT for effectiveness
T- skin tissue necrosis, teratogenic |
|
|
Term
|
Definition
tPA (alteplase), reteplase (rPA), tenecteplase (TNK-tPA)
Aid conversion of plasminogen to plasmin, which cleaves thrombin and fibrin clots
Inc PT, PTT, no change in platelet count
Contraindicated in patients with active bleed
Tx tox- aminocaproic acid |
|
|
Term
|
Definition
Irreversible inhibits COX 1 and 2 by covalent acetylation
Inc BT, dec TXA2 and prostaglandins
T- gastric ulceration, tinnitus
OD- resp alkalosis and metabolic acidosis |
|
|
Term
|
Definition
Clopidogrel, ticlopidine, prasugrel, ticagrelor
Inhibit platelet aggregation by blocking ADP receptors, which inhibits the Gp2b/3a binding fibrinogen
For coronary stenting
T- neutropenia |
|
|
Term
|
Definition
PDE 3 inhibitors
Inc cAMP in platelets, inhibiting aggregation
For prevention of stroke or TIAs
T- nausea, HA, facial flushing, hypotension |
|
|
Term
|
Definition
Abciximab, eptifibatide, tirofiban
Prevents aggregation
Acute coronary syndromes, percutaneous transluminal coronary angioplasty |
|
|
Term
|
Definition
Inhibits dihydrofolate reductase
Dec dTMP, dec DNA and dec protein synthesis
RA, psoriasis, ectopic pregancy
T- myelosuppression (reversible with leucovorin), fatty change in liver |
|
|
Term
|
Definition
pyrimidine analog, covalently complexes folic acid
Inhibits thymidylate synthase, dec dTMP, dec DNA and protein synthesis
Colon cancer, basal cell carcinoma
T- myelosuppression, photosensitivity |
|
|
Term
|
Definition
Pyrimidine analog, inhibition of DNA polymerase
T- leukopenia, thrombocytopenia, megaloblastic anemia |
|
|
Term
|
Definition
Purine analogs, dec de novo purine synthesis
Acitvated by HGPRT
T- BM, GI, liver, metabolized by xanthine oxidase, thus Inc toxicity with allopurinol |
|
|
Term
|
Definition
Intercalates DNA
Wilms tumor, Ewingssarconoma, rhabdomyosarcoma
T- myelosuppression |
|
|
Term
| Doxorubicin, daunorubicin |
|
Definition
Generates free radicals, non covalently intercalates DNA causing breaks
T- cardiotox (dilated cardiomyopathy), myelosuppression, alopecia
Use dexrazoxane to prevent cardiotox |
|
|
Term
|
Definition
Free radical formation, breaks in DNA strands
T- pulmonary fibrosis, skin changes |
|
|
Term
| Cyclophosphamide, ifosfamide |
|
Definition
Cavalently X link DNA at guanine N-7
Requires bioactivation by liver
T- myelosuppression, hemorrhagic cystitis (pref with mensa) |
|
|
Term
|
Definition
Require bioactivation, crosses blood brain barrier
*Brain tumors
T- CNS toxicity |
|
|
Term
|
Definition
Alkylates DNA
CML, used to ablate patients BM before BM transplantation
T- pulmonary fibrosis, hyperpigmentation |
|
|
Term
|
Definition
Alkaloids that bind to tubulin in M phase and block polymerization, so mitotic spindle cannot form
T- neurotoxicity (vincristine), BM suppression (blast) |
|
|
Term
|
Definition
Hyperstabilizes M phase microtubules so spindle cannot break down
Ovairan and breast carcinomas
T- Myelosuppresion, hypersensitivity |
|
|
Term
|
Definition
Cross links DNA
T- Nephrotoxity and acoustic nerve damage
Prevent hearing damage with amifostine and chloride diuresis |
|
|
Term
|
Definition
Inhibits topoisomerase 2, leading to inc DNA degradation
T- myelosuppression, GI irritation, alopecia |
|
|
Term
|
Definition
Inhibits ribonucleotide reductase, dec DNA synthesis
T- BM suppression, GI upset |
|
|
Term
|
Definition
May trigger apoptosis
Most commonly used glucocorticoid in cancer chemotherapy
CLL, non hodgkin lymphomas
T- cushing like symptoms, immunosuppression |
|
|
Term
|
Definition
SERMs, block the binding of estrogen to ER pos cells
T- partial agonist of endometrium, which inc risk of cancer (only in tamoxifen) |
|
|
Term
|
Definition
Monoclonal Ab against HER-2, a tyr kinase
T- cardiotoxicity |
|
|
Term
|
Definition
Bcr-Able tyr kinase inhibitor
CML
Fluid retention |
|
|
Term
|
Definition
Against CD20, B cell neoplasms
Non hodgkins lymphomas, RA |
|
|
Term
|
Definition
Inhibitr of B-Raf kinase with V600E mutation
Metastatin melanoma |
|
|
Term
|
Definition
Against VEGF
Solid tumors to prevent angiogenesis |
|
|
