Blood and Immunology Flashcards
DDx microcytic anemia (3)
- Iron deficiency, 2. Thalassemia, 3. Chronic inflammation
Normocytic anemia DDx
Blood loss, immune hemolytic, spherocytosis, sickle cell, renal disease
Macrocytic anemia DDx
Folate def, B12 def, liver disease, hypothyroid, neoplasms, aplastic anemia,
Risk factors for Hodgkin lymphoma
EBV, immunosupression/HIV, autoimmune diseases
signs of acute hemolytic transfusion reaction
Fever, hemoglobinuria, hypotension, DIV, evidence of hemolysis, complement activation
Signs of hemolysis on labs
Low haptoglobin and high LDH
Delayed hemolytic transfusion reaction causes hemolysis where?
Extravascular hemolysis in the reticuloendothelial system (spleen)
Reticuloendothelial system organ is
Spleen
Preventing hemolytic disease of the fetus
Give rh immune globulin to d negative moms before they form antibodies
Neonatal Alloimmune thrombocytopenia mechanism
Antibodies vs platelet antigens in fetus leading to severe bleeding complications at time of delivery
Transfusion related acute lung injury (TRALI)
Antibody mediated reaction from donor units that activate neutrophils in the recipient causing inflammatory damage to the lungs
Transfuse if Hgb is less than
70
How many platelets in a donor pack
30x10^10
Shelf life of platelets
7 days
Female donor plasma is used to make
Albumin and IvIg
Male plasma is used for
Transfusion, cryosupernatant, cryoprecipitate
INR threshold for transfusion
INR 1.6
Immune mediated transfusion complications
Hemolytic, febrile/allergic, TRALI, PTP, TA-GVHD, alloimmunization
Non immune mediated hazards of transfusion
Sepsis, non-immune hemolysis, mistransfusion, TACO, coagulopathic complications, iron overload
Pattern recognition receptors are a component of which immune system
Innate immune system
B and T cells are associated with which type of immunity
Adaptive
Antigen receptor type on B cells
Ig
Central tolerance immune system happens in
Thymus and bone marrow
Autoantibodies associated with rheumatoid arthritis
Rheumatoid factor, antibodies against citrullinated protein antigens (ACPA)
MHC presents the antigen to
T cell receptors
CD28 is located in
The T cell
HLA-B27 is associated with
Ankylosing spondylitis
Kawasaki disease is a
Medium vessel vasculitis affecting children
In SLE, autoantibodies are to
The cell nucleus
SLE arthritis is
Non erosive in a distribution like rheumatoid arthritis
SLE manifestations (renal)
Lupus nephritis: persistent proteinuria, cell casts, hematuria, pyuria, hypertension, increased creatinine
CNS manifestations neurologic
Stroke, vasculitis, seizures, psychosis
Hematologic disorders of SLE
Hemolytic anemia, leukopenia, thrombocytopenia
Autoantibodies specific to SLE
Anti-dsDNA
ANA+ in what % of SLE cases
> 95%
The presentation of SLE is
Heterogenous - some kind of antibodies, and inflammation
Drug classes used to treat SLE include
Antimalarials, corticosteroids, immunosuppressants, and biologicals
SLE stands for
Systemic lupus erythematosus
Type I hypersensitivity is how fast?
Immediate
Type I hypersensitivity mediated with Ig…what?
IgE
Allergic reactions are usually which type of hypersensitity
Type I, IgE mediated q
Type II hypersensitivity involves which Ig?
IgM or IgG
Thrombocytopenia and hemolytic anemia are which type of hypersensitivity
Type II
Immune complexes depositing in vascular beds is what type of hypersensitivity reaction
Type III
Which type of hypersensitivity reactions are T cell activation and involve induction of inflammation through macrophages and inflammatory cytokines like TNF
Type IV hypersensitivity
Which type of hypersensitivity for rheumatoid arthritis, multiple sclerosis, and IBD, type I diabetes
Type IV
Sjogrens occurs secondary to
RA, SLE, Scleroderma, myositis
Sjogrens on bloodwork
ANA+, anti-Ro or anti-La, RF+, anemia of chronic disease
In cryoglobulinemia, autoantibodies
Precipitate at low temperatures and cause vasculitis in the small vessels by IC deposition
Malignancy in Sjogrens
44x fold increased risk for lymphoma, usually MALT
MALT stands for
Mucosa associated lymphoid tissue
Localized scleroderma presents s
Morphea and linear scleroderma
Raynaud’s phenomenon is
Exaggerated vasoconstrictive response to cold or stress leading to colour change in digits
Raynauds is common in what demographic?
Young women
Raynauds - what lifestyle changes for treatment? What medication?
Stop smoking, keep warm. Treat medically with vasodilator.
Vasodilator classes for raynauds treatment (3)
CCB, ARBs, nitrates
Lab work in scleroderma
ANA+ in 50-80%, anticentrometere + 80%, frequently anemic
Treating scleroderma
Treat specific organ manifestations
What is elevated in polymyositis
CK, CPK, 50% ANA +
Dermatomyositis has a characteristic rash consisting of
Gottren’s papules, heliotrope rash, shawl sign, nailfold capillary changes
Palpable purpura is
Non blanching, palpable, lower extremity predominant
Lab work glomerulonephritis
High creatinine, proteinuria, RBC casts
Mononeuritis multiplex
Ischemia of vessels supplying nerves leads to periopheral nerve damage
Large vessel vasculitis includes
Takayasu’s arteritis, giant cell arteritis
Stenosis of the aorta and major branches is called
Takayasu’s arteritis
GCA affects people aged
Older than 50
Jaw claudication in GCA is due to
Ischemia of the muscles of mastication
Vision changes in GCA due to
Retinal artery occlusion
Treatment of GCA is with
High dose steroids, and low dose ASA
Areas affected by polymyagia rheumatica
Pectoral and pelvic girdles
Bloodwork for polymyalgia rheumatica
High ESR CRP, anemia of chronic disease
Treating polymyalgia rheumatica
Low dose steroids
What antibody has high specificity for GPA
C-ANCA (vs PR-3)
Churg strauss syndrome is also known as
Eosinophilic granulomatosis with polyangitits
Characteristic of Eosinophilic granulomatosis with polyangiitis
Systemic vasculitis with chronic rhinosinusitis, nasal polyps, asthma, and prominent eosinophilia
When is eosinophilic granulomatosis with polyangitis typically diagnosed?
Adulthood
Signs of IgA deficiency
Typically asymptomatic, though recurrent bacterial sinopulmonary infections can occur
Severe combined immunodeficiency usually results from
X linked mutations
Signs of severe combined immunodeficiency
Recurrent severe infections, poor growth, chronic diarrhea, death by 12 months without stem cell transplant
Treating severe combined immmunodeficiency
Hematopoetic stem cell transplant
Typical presentation of myasthenia gravis
Fluctuating, fatiguable muscle weakness that worsens with repetitive motion and improves with rest
MG is caused by
Autoantibodies directed against nicotinic AcH receptors at the NMJ
Achalasia occurs due to
The desecration of Neurons within the myenteric plexus
MS is characterized by
Inflammatory demyelination of axons in the CNS
Acute urticaria is caused by
Mast cell activation in the superficial dermis
Initial management of acute urticaria includes
Second generation H1 blocker eg. Cetirizine
Photosensitive rash along with multisystemic symptoms is suspicious for
SLE
ANA assay is highly sensitive for
SLE
How do you test for SLE
ANA assay
High reticulocyte count anemia is usually due to
Acute bleeding or hemolysis
The most common nutritional deficiency in infants is
Iron deficiency
In thalassemia the iron and ferritin levels are
Normal or high
In thalassemia, reticulocytes and total bilirubin count is
Increased due to hemolysis
TSAT is calculated by
serum iron / TIBC
What type of anemia is caused by folate and vitamin b12 deficiency
Megaloblastic anemia
High MCV, high MCH, normal hemoglobin
Megaloblastic anemia
Inherited defect in RBC membrane that presents with hemolytic anemia, jaundice, splenomegaly
Hereditary spherocytosis
Hemophilia A has absent or low factor
VIII 8
Hemophilia B has low or absent factor
IX 9
Phenytoin can cause what kind of anemia?
Megaloblastic anemia due to folic acid deficiency
Patients on phenytoin should be supplemented with
Folic acid
Medications that commonly cause megaloblastic anemia include
Methotrexate, TMP, phenytoin
Risk factors for MS
Female, Caucasian, low vitamin D levels, smoking
Vitamin deficiency that can lead to pancytopenia with normal coagulation studies
Vitamin B12 deficiency
Treating stroke in sickle cell disease
Exchange transfusion - increases carrying capacity, improves hyper-viscosity, limits further occlusion
What kind of anti coagulant is appropriate for treating patients with ischemic stroke due to atherosclerosis, small vessel disease, or thromboembolism?
Antiplatelet agents
Allergic contact dermatitis is which type of hypersensitivity reaction?
Type IV, cell mediated
Treating allergic contact dermatitis
Topical corticosteroids and avoidance of the allergen