Path Flashcards
How to monitor activity of SLE?
C3 and C4 levels
How to monitor patient with HIV?
WCC and differential.
Viral load is determined by PCR. Used to detect viral RNA to determine if virus is replicating
Antibody to diagnose someone with systemic sclerosis/scleroderma?
anti-toposiomerase aka anti-SCL-70
Management of flare of ANCA-assoc. vasulitis?
ie Wengeners, PAN, microscopic polyangitis
Induce remission with steroids and cyclophosphamide (or rituximab)
Maintain relapse by weaning off steroids and switch CYC to AZA or MTX (or continue rituximab)
Wean off biologic
Management of rheumatoid arthritis?
Induce remission with steroids and methotrexate.
Can add in a biologic like etanercept/infliximab/tocilizumab (based on what is cheapest) if inadequate control.
Rituximab is 3rd line if other dmards fail
Initial treatment is for 6 months then wean down dose as symptoms reduce. Increase if theres another flare.
Monotherapy for RA?
can use Adalimumab, etanercept, certolizumab or tocilizumab for monotherapy if methotrexate is contraindicated eg pregnancy or not tolerated
Systemic management of psoriasis?
Methotrexate or ciclosporin are first choice non-biologic systemic drugs. Monitor for hepatotoxicity with methotrexate.
If not responding to both, consider anti-TNF (adalimumab, etanercept, infliximab 2nd line) or anti-IL12 (ustekinumab)
Treatment of chronic granulomatous disease?
prophylactic trimethoprim and itraconazole.
INF-gamma for immunomodulation
Biologic used in treatment of osteoporosis?
Denosumab
how to manage acute attack of swelling in C1 inhibitor deficiency?
IV C1 inhibitor.
Chronically reduce freq. with tranexamic acid or regular C1 injections
What is the difference between H1 and H2 antagonists?
H1 are used to treat allergic reactions examples cyclizine, loratadine, promethazine, cetirazine
H2 are used to reduce gastric acid secretions egs cimetidine, ranitidine
Which HIV antigen do neutralising antibodies bind to?
envelope glycoprotein gp120 and gp41
Very specific to a certain region on HIV1 viruses
Which HIV antigens are non-neutralising antibodies produced against?
Most Env (viral envelope protein) are non-neutralising
Anti p24 (caspid protein) are non-neutralising
NB, body can also produce non-neutralising antibodies to monoclonals eg anti-TNFs like etanercept
What coreceptors on T-cells are required for viral entry?
CXCR4 and CCR5
What drugs are used for HIV antiviral therapy and how do they work/what is the target?
Nucleoside reverse transcriptase inhibitors (nucleoside analogues so competitive inhibition)
Non-nucleoside reverse transcriptase inhibitors (non-competitive inhibition)
Integrase inhibitors inhibit integration of viral DNA into host cell DNA
Protease inhibitors block viral enzyme which cleaves proteins necessary to form mature virions
6 month old with failure to thrive and recurrent infections. T cells undetectable and B cells normal, reduced antibody levels.
X-linked SCID.
Defect of IL2 receptor means no t cells and no maturation of B cells
Young person with recurrent episodes of meningococcal sepsis - what is likely immunodeficiency?
Complement deficiency. Def of any in the terminal pathway (C3,5,6,7,8,9) predispose to meningococcus and pneumococcus
What is the likely deficiency if CH50 test only is abnormal?
C4 most likely. Could be C1 or 2
What is the likely deficiency if AP50 test only is abnormal?
Factor B, I or P
Predisposes to encapsulated bacterial infection
Hib, pneumococc, meningococc, GBS, klebsiella, salmonella, e.coli
What vaccines should be avoided in the immunocompromised?
Live vaccines: Smallpox Yellow fever Typhoid Chickenpox MMR (still give in HIV unless severely immunocompromised) BCG oral polio (sabin)
What vaccine is given to asplenic patients and repeated every 5 years?
pneumovax
What vaccine is part of routine schedule but avoid in immunodeficiency?
MMR
ok in HIV or diGeorge if no evidence of severe immunocompromise
Which vaccine is a component vaccine consisting of viral haemagglutinin?
influenza
What are the target INRs for warfarin therapy?
2.5 (2-3) for most indications eg VTE, AF, biologic prosthetic valve etc
A target of 3 (2.5-3.5) is indicated for a mechanical aortic valve
A target of 3.5 (3-4) is indicated for a VTE while on anticoagulants or a mechanical mitral valve
warfarin overdose, INR 5-8 with no bleeding
stop warfarin restart when under 5
Warfarin overdose INR 5-8 with minor bleed
Stop warfarin, slow Vit K IV. Restart warfarin when INR
Warfarin overdose, INR>8 with no bleed
Stop warfarin, seek specialist advice, Oral Vit K. Repeat in 24hr if INR still high. Restart warfarin if INR
Warfarin overdose, INR>8 with minor bleeding
Stop warfarin. Arrange admission for IV Vit K. Daily INR check. Restart warfarin when INR
Warfarin overdose (INR above therapeutic range) and major active bleeding
Stop warfarin, Urgent admission. IV Vit K and dried prothrombin complex (Factors II, VII, IX, X)
Immediate transfusion reaction where patient has a high fever
Bacterial contamination. If temp rises above 39C or by more than 2C
Immediate transfusion reaction where patient has anaphylaxis. Has had another transfusion years ago
IgA deficiency
Patient suffers acute sever trauma, transfused with O- blood as emergency. Then loses consciousness a few mins after transfusion
Most likely internal hemorrhage
Immediate transfusion reaction, sudden onset dyspnoea with hypotension
TRALI
Immediate transfusion, sudden onset dyspnoea. Normotensive or hypertensive. Raised JVP
TACO -(fluid overload)
Immediate transfusion reaction. Fevers, chills, pain at site of transfusion, nausea/vomiting, BP drop, dark urine.
Confusion or possibility for error in samples sent
ABO incompatibility - acute haemolytic reaction. STOP transfusion. IV saline and diuretics. Keep blood for testing
Immediate transfusion reaction. Fevers, chills. Temp rise by less than 1C
Febrile reaction. Give paracetamol. Dont stop transfusion
Most common hereditary thrombophilic disorders
Factor V Leiden
Prothrombin G20210A
Rarer hereditary thrombophilias
Antithrombin III deficiency,
Protein C deficiency
Protein S deficiency
Recurrent miscarriages, hypercoagulable, assoc with SLE
Antiphospholipid syndrome
Pregnant woman develops seizures. Shes anaemic and jaundiced. Schistocytes seen on blood film. Febrile with neuro syx. PT and APTT normal
TTP/HELLP syndrome.
NOT DIC b/c of neuro symptoms and normal coagluation cascade (dysregulated in DIC)
The clots in TTP are aggregated platelets cf DIC where they are fibrin clots
Pregnant woman. Chorioamnionitis. Becomes septic. Bruises form. PT and APTT prolonged.
DIC
30/40 pregnant lady. Antenatal care all normal. Develops nosebleeds/bleeding gums/easy bruising. Otherwise well.
Gestational thrombocytopenia.
Occurs in mid 2nd trimester onwards
May present asymptomatically with diagnosis on routine FBC (eg 28-week second screed)
Child with recurrent infection. Normal CD8 levels and absent CD4
Bare Lymphocyte Syndrome type 2
Type 1 is rarer. Deficient in CD8 and normal CD4
Boy presents at 1 yr with recurrent infections and failure to thrive. High levels of IgM. Low IgA and IgG. What is the deficiency?
CD40L deficiency. Causes Hyper IgM syndrome
CD40L def is X-linked recessive. Other types can be autosomal recessive
Person with normal Hb and positive sickle solubility test
Sickle cell trait
Patient with low Hb and positive sickle solubility test
Sickle cell anaemia
trait would have normal Hb
African man becomes jaundiced and anaemic after taking primiaquine and chloroquine for malaria
G6PD deficiency. X-linked. Drug trigger
Older man with splenomegaly, fatigue and anaemia. Peripheral film shows polychromasia and spherocytes
Hereditary spherocytosis
Polychromasia = reticulocytosis
55yr old woman. Incidental finding of isolated platelet count >600. On further questioning, she has noted gum bleeding and headaches/dizziness. She has mild splenomegaly.
Essential thombrocythema.
Treat with aspirin (antithrombosis) Anegrelide (inhibits platelet formation) and hydroxycarbamide (anitmetabolite and BM suppression)
3-month old admitted for pneumonia. White cells are up, Platelets are 510. What is the cause of the thrombocytosis?
Reactive (secondary) thrombocytosis
70 yr old man is feeling increasingly tired. Bloods show he is anaemic and has raised platelets and neutrophilia
CML
A vegetarian lady presents feeling tired. She has a low Hb and platelets are 470
Iron deficiency raises platelets
An afrocarribbean boy has anaemia and a raised platelet count and a palpable spleen
Sickle cell causes hyposplenism, which can cause a reactive thrombocytosis
A man has a routine hernia repair. After the surgery his platelets go up
Reactive thrombocytosis secondary to surgery
Infection in pregnancy: what is a flagellate protozoan that can cross the placenta?
Toxoplamsa gondii
Infection in pregnancy - what is an STI previously beleived to only cross the placenta in the third trimester?
syphilis
Infection in pregnancy that can be teratogenic if mother eats unpasteurised cheeses?
Listeria
Normal maternal commensal that can cause neonatal sepsis
GBS
STI that can cause neonatal conjunctivitis
Chlamydia or gonorrhoea