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resexuwe's version from 2017-08-07 17:11

Biochemistry Cont..

Question Answer
How does Scleroderma present?Tight skin
How does Ehlers Danlos present?Hyperstretchable skin
How does Marfan present?Hyperextensible joints, arachnodactyly, wing span longer tan height, aortic root dilatation, aortic aneurysm, mitral valve prolapse, dislocated lens from bottom of eye -> Look up
How does Homocystinuria present?Dislocated lens from top-> look down
How does kinky hair disease present?Hair looks like cooper wire (Cu deficiency)
How does scurvy present?Bleeding gums, bleeding hair follicles
How does Takayasu arteritis present?Asian female with very weak pulse
How does Osteogenesis imperfecta presentShattered bones, blue sclera
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Hematology

Question Answer
BabesiosisInfection with a protozoan that activates complement
What is a Neutrophil?The phagocyte (has anti microbials, most abundant)
What is an Eosinophil?The parasite destroyer, Allergy inducer
What is a basophil?The allergy helper (IgE receptor -> Histamine release)
What is a Monocyte?The destroyer -> MP (hydrolytic enzymes, coffee-bean nucleus)
What is a lymphocyte?The warrior -> T,B,NK cells
What is a Platelet?The clotter (no nuceli, smallest cells)
What is a blast?Baby hematopoietic cell
What is a band?Baby neutrophil
What does high WBC and high PMNs tell you?Stress demargination
What does High WBC and <5% blasts tell you?Leukomoid reaction, seen in burnpts (extremem demargination looks like leukemia)
What does high WBC and >5% blasts tell you?Leukemia
What does high WBC and bands tell you?Left shift -> have infection
What does High WBC and b cells tell you?Bacterial infection
What diseases have high eosinophils?NAACP Neoplasm(lymphoma), Allergy Asthma, Addisions disease(no cortisol -> relative eosinophilia), Collagen vascular disease, Parasites
What disease have high monocytes (>15%)?STELS Syphilis(Chancre, rash, warts), Tb(hemoptysis, night sweats), Ebv(teenager sick for a month) , Listeria(baby who is sick), Salmonella(food)
What do High retics (>1%) tell you?RBC being destroyed peripherally
What do low retics tell you?Bone marrow not working right (decrease production)
What is Poikilocytosis?Different shapes
What is Anisocytosis?Different sizes
What is the RBC life span?120 days
What is the platelet lifespan?7 days
What does penia tell you?Low levels (usually due to virus or drugs)
What does cytosis tell you?High levels
What does cythemia tell you?High levels
What is the difference between plasma and serum?Plasma- No RBC / Serum- No RBC or fibrinogen
Only leukemia that increases PT & PTTAML
TB markerCD 4 & CD 14
What is Chronic granulomatous disease?NADPH oxidase deficiency -> recurrent Staph/Aspergillus infection (Nitroblue tetrazolium stain negative)
What does MPO deficiency cause?Catalase + infections
What is Chedial Higashi?Lazy leukocyte syndrome -> Lysosomes, are slow to fuse around bacteria
What organ can RBCs if the long bones are damaged?Spleen -> splenomegaly
What causes a shift to the right in the Hb curve?All CADETSs face right Increase CO2, Acid/Altitude, 2,3-dpg, Exercise, Temperature
How does CO poison Hb?Competitive inhibitor of O2 on Hb -> cherry red lips, pinkish skin hue
How does Cyanide poison Hb?Non-competitive inhibitor of O2 on Hb -> almond breath
What is MetHb?H w/FE3
What is Acute intermittent porphyria?Increase Porphyrin, urine delta-ALA, porphobilinogen -> abdominal pain, neuropathy, red urine
What is Porphyria Cutanea Tarda?Sunlight -> skin blisters w. porphyrin deposits, woods
What is Erythrocytic Protopoprhyria?Prphyria cutanea tarda in a baby
What is a sickle cell trait?Hetercygous HbS -> painless hematuria, sickle with extreme hypoxia (Cant be a pilot, fireman, diver)
What is a Sickle cell disease?Homocygous HbS -> (Bglu6 -> Val) -> Vasoocclusion, necrosis, dactylitis (painful fingers/toes) at 6 month, protects against malaria
What is Hb C disease?(Bglu6-lys) still charged -> no sickling
What is a-thalassemia?1.deletion-Normal, 2.Deletions "trait" - Microcytic anemia, 3.Deletions- hemolytc anemia, Hb h-b4, 4.deletions- hydrops fetalis, Hb bart-y4
What is B thalassemia?1.deletions "b minor" - HbA2 and HbF, 2.deletions "trait/intermedia.major" -> only HbA2 and HbF -> Hypoxia at 6 month
What is Cooleys anemia?See w/ B thalassemia major (no HbA -> excess RBC production); baby making blood from everywhere -> frontal bossing, hepatosplenomegaly, long extremities
What is Virchows Triad?Thrombosis risk factors -> 1. Turbulent blood flow "slow" 2. Hypercoagulable "sticky" 3.Vessel wall damage"escapes"
What does acute hypoxia cause?Shortness of breath
What does chronic hypoxia cause?Clubbing of fingers.toes
What is intravascular hemolysis?RBC destroyed in blood vv -> low haptoglobin (bbinds free floating)
What is extravascular hemolysis?RBC destroyed in spleen (problem w/ RBC membrane) -> splenomegaly
What enzymes need lead? (Pb)Delta-ALA dehydratase, Ferrochelatase
What does EDTA bind?X2+
What disease has a smooth philthrum?Fetal alcohol syndrome
What disease has long philthrum?Williams
What disease has sausage digits?Pseudo-hypoparathyroidism, psoriatic arthritis
What disease has 6 fingers?Trisomy 13
What diease has 2 jointed thumbs?Diamon blackfan & Edqards
What disease has painful fingers?Sickle cell disease
What are the Microcytic Hypochromic anemias? FAST LeadFe deficiency, Anemia of chronic disease, Siderblastic anemia, a-thalassemia, b-thalasssemia, Pb posioning
Fe deficiencyIncrease TIBC, MENSES, GI bleed, Koilonychia
Anemia of chronic diseaseDecrease TIBC
Siderblastic anemiaDecrease d-ALA synthase, blood transfusions
a-thalassemiaAA, Asians, Chr 16 deletion
b-thalasssemiaMediterraneans Chr 11 point mutation
Pb posioningDecrease d-ALA dehydratase, Decrease ferrochelaase, xrayblue line, eating old paint chips
What are the megaloblastic anemias?Vit B12 deficiency, Folate deficiency, Alcohol
Vit b12 deficiencyTapeworms, vegans, type A gastritis,pernicious anemia
Folate deficiencyOld food, glossitis
Alcohol Fetal alcohol syndrome -> smooth philthrum, stuff doesnt grow
What are the intravascular hemolytic anemias?IgM -> G6PD deficiency, Cold autoimmune
G6PD deficiencySulfa drugs, moth balls, fava beans, sudden dropin Hb
Cold autoimmuneMononucleosis, mycoplasma infections, RBC agglutination
What are the Extravascular Hemolytic anemias?IgG -> Spherocytosis, warm autoimmune, Paroxymal cold autoimmune, Sickle cell anemia
SpherocytosisDefective spherin or ankyrin, +osmotic fragility test
warm autoimmuneAnti-Rh Ab, dapsone, PTU, anti-malarials, sulfa drugs
Paroxymal cold autoimmuneBleeds after cold exposure, Donath-Landsteiner Ab
Sickle cell anemiaCrew haircut xray, avascular necrosis of femur, short fingers
What are the production Anemias?Diamond black fan Pure red blood aplasia, Aplastic anemia
Diamond black fanNo RBCs, 2 Joined thumbs
Aplastic AnemiaPancytopenia, autoimmune, benzene, AZT, CAM, radiation
What is Basophilic Stippling?Lots of immature cells, Increase mRNA lead poisoning
What is a bite cell or Basket cell?Unstable Hb inclusions g6pd deficiency
What is Burr cell or Echinocyte?Pyruvate kinase deficiency, liver disease, Post splenectomy
What is Cabots ring body?Vit B12 deficiency, Lead poisoning
What is Doehle body?PMN leukocytosis (infection, steroids, tumor)
What is Drepanocyte?Sickle cell anemia
What is a helmet cell?Fragmeneted RBC (hemolysis- DIC, HUX, TTP)
What is a heinz body?Hb precipates and sticks to cell membranes(G6pd)
What is a Howell Jolly body?Spleen or bone marrow should have removed nuclei fragments (hemolytic anemia, spleen trauma, cancer)
What is Pappenheimer body?Fe ppt inside cell (Sideroblastic anemia)
What is Pencil cell or cigar cell?Fe deficiency anemia
What is a rouleaux formation?Multiple myeloma
What is Schistocyte?Broken RBC (dic, artifical heart valves)
What is a Sideroblast?Macrophages pregnant w/Fe (genetic or multiple transfusions)
What is a spherocyte?Old Rbc
What is a spur cell or acanthocytes?Lipid high in serum
What is a stomatocyte?Liver disease
What is a target cell-> Codocyte?Less Hb (thallasemias or Fe deficiency)
What is a tear drop cell-> Dacrocyte?RBC's squeezed out of marrow(hemolytic anemia, bone marrow)
What is the clotting cascade?How you stop bleeding?
What are the vitamin K clotting factors?10,9,7,2 Protein C & S
What do platelet problems cause?Bleeding from skin and mucosa
What do clotting factor problems cause?Bleeding into cavities
What causes increased PTT and bleeding time?von Willebrand disease and Lupus
What is bernard Soulier?Baby w/ bleeding from skin and mucosa, big platelets (low GP1b)
What is Glanzmanns?Baby w/ bleeding from skin and mucosa (low GP2b3a)
How does factor 13 deficiency present?Umbilical stump bleeding (1st time baby has to stabilize a clot)
What is factor V leiden?Protein C cant break down factor 5 -> more clots
How does von Willebrand disease present?Heavy menstrual bleeding
What are the types of VWD?Type 1(AD)- DECREASE vWF production / Type 2(AD)- Decrease vWF activity(+Ristocetin aggregation test) / Tpe 3(AR)- No vWF
What is Hemophilia A?Defective Factor 8(<40% activity) -> bleed into cavities (head, abdomen, etc)
What is Hemophilia B?Factor 9 deficiency -> bleed into joints (knee, etc)
What disease have low LAP?CML, PNH
What has high LAP?Leukemoid reaction
What is the difference between acute and chronic leukemias?Acute- started in bone marrow, squeezes RBC out of marrow / Chronic- Started in periphery, not constrained -> will expand
What is the difference between myeloid and lymphoid leukemias?Myeloid- Increase RBC, WBC, Platelets, MP,(decrease lymphoid cells)-> bone marrow biopsy / Lymphoid- Increase NK, T, B cells (ndecrease myeloid cells) -> do lymph node
What defines ALL?<15 YR male bone pain, PAS stain+, TdT+
What defines AML?15-30 yr males, sudan stain, Auer rods
What defines AML?15-30YR old females, Sudan stain , Auer rods
What defines CML?30-50 yr females, t(9,22) "Philadelphia chromosome", bcr-abl, Decrease LAP
What defInes CLL?>50 yr males with lymphadenopathy, "Soccer ball" nuceli, smudge cells
What defines hodgkin lymphoma?EBV, may have Reed sternberg cells
What are the B cell non-hodgkin lymphomas?Follicular -> t(14,18), bcl 2 / Burkitt -> ~t(8,14), c-myc, starry sky MP, American kids(abdominal mass), African kids(jaw mass)
What are the T cell non-hodgkin lymphomas?Mycosis Fungoides -> Total body rash / Sezary syndrome -> Cerebreform
What is Polycythemia Vera?Hct >60%, decrease EPO, budd chiari plethoric "pruritis after bathing"
What is Essential Thrombocythemia?Very high platelets, stainable Fe, decrease c-mpl
What is Myelofibrosis?Megakaryocytes, fibrotic marrow -> Teardrop cells, extramuscular hematopoiesis
What are plasma cytomas?Produce lots of Ab
What is Waldenstrom Macroglobulinemia?IgM, hyperviscous
What is Monoclonal Gamoopathy of undetermined significance?Old person w. gamma spike?
What is Multiple Myeloma?Serum M prot (IgG), urine Bence-jones protein, rouleaux, punched out lesions
What is heavy chain disease?Increase IgA, infiltration of bowel wall
What is Histocytosis X?Kid w/ eczema, skull lesions, diabetes insipidus, exopthalmos
What does the Coombs test tell you?Ab involved agianst RBC
What does the direct Coombs test tell you?On surface -> hemolytic anemias
What does the indirect Coombs test tell you?In serum
What is type and cross?You know you can use that blood, save it for specific pt
What is type and match?Type it and wait
What is forward typing?Uses Ab to detect Ag Fabulous
What is backward typing?Uses Ag to detect Ab
What does blood type A tell you?Have the A antigen
What does blood type O tell you?Have no antigens, universal donor
What does blood typa AB tell you?Have both antigens, universal reciptent
What does Rh+ tell you?Has D antigen
What does Rh- tell you?Does not have D antigen
What is the hemolytic disease of the newborn?Rh- Moms placenta tears, 100cc baby's blood sees Mom/produces Ab, attacks fetus
What is RHOGAM?Anti-D IgG
When do you give RHOGAM?1ST dose after delivery of 1st baby / 2ND dose week 28 of any subsequent pregancy
What is the most common transplant?Blood
What is synergenic transplant?Twin to twin
What is Autograft?Self to self
What is and Allograft?Human to human transplant
What is a Xenograft?1 specie to another specie
What is Hyperacute rejection?Within 12 hrs preformed Ab
What is Acute rejection?4 days to years later Tcells, MP
What is Chronic rejection?>7days Fibroblasts
What is Graft vs Host disease?Bone marrow transplants reject (Tk, MP)
What are the immunopriivileged sites?No lymphatic flow-> No Ag -> Easy to transplant brain, cornea, thymus, testes
No History of bleeding disroder & Increased PTT is due to abnormalKallikrenin formation
Sarcoidosis Lab valuesHypercalcemia, Hyponatremia
PANTransmural inflammation of arteries -> Muscle biopsy will show segmental ischemic necrosis
In carbon monoxide, Increased Carboxyhemoglobin meansOxygen now has affinity for carboxyhemoglobin
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