hematology/immunology Flashcards
Idiopathic Thrombocytopenia Purpura (ITP) labs
- Normal PT and PTT
- blood smear - enlarged/immature/decreased platelets
- Thrombocytopenia with normal RBC/WBC
vitamin K deficiency labs
PT/INR elevated and normal PTT
decreased factors II, VII, IX and X
von willebrand disease labs
increased bleeding time, increased PTT
- normal platelets
- decreased factor VIII
- decreased vW antigen
what symptom in B12 deficiency is not found in folate deficiency
neurological sx
sx same in B12 and folate deficiency
- smooth, sore tongue with atrophy of papillae
labs same for alpha thalassemia and beta thalassemia minor
- decreased MCV, Hb and Hct
- Increased RBC count
- Normal RDW, ferritin
alpha thalessemia diagnosis
DNA analysis with alpha gene probes is the only way to diagnose
alpha thalessemia reticulocytes
normal
beta thalassemia major reticulocytes and RDW
increased
diagnosis of beta thalassemia
Hb electrophoresis
polycythemia vera mutation of what gene
JAK2 gene on chromosome 9
polycythemia vera EPO and SAO2 labs
decreased EPO, normal SAO2 (O2 saturation)
polycythemia vera labs
- increased RBC count and RBC mass
- decreased EPO, normal SAO2
diagnosis of polycythemia vera
3 major criteria OR first 2 major and 2 minor
- Major criteria:
1. increased red cell mass
2. no cause of secondary erythrocytosis
3. palpable splenomegaly
4. JAK2 gene - Minor:
1. Absolute leukocytosis
2. thrombocytosis
3. leukocytosis
4. low serum EPO
4. Bone marrow Bx reveales myelosis w/ megakaryocytes
secondary polycythemia vera can be due to
- high altitude
- COPD
- CVD with R->L shunt
- sleep apnea
- carbon monoxide exposure
treatment of polycythemia vera
- phlebotomy
- interferon alpha
disseminated intravascular coagulation labs
- increased PT and PTT
- decreased fibrinogen
- thrombocytopenia
- increased bleeding time
- increased D-dimer
- normocytic anemia
hemophilia labs
- prolonged PTT, normal INR
anti-HIV antibodies are detectable when
3 weeks to 3 months after infection
initial screening test for HIV
ELISA (detects anti-gp120 Abs, 100% sensitivity)
Confirmatory test for HIV
western blot
What is CD4 T cell count used for in HIV
monitoring
What is HIV viral load used for in HIV
- detection of dividing virus, marker of disease progression
Treatment of HIV
Zidovudine (nucleoside analog reverse transcriptase inhibitors)
Ankylosing Spondylitis and Reactive Arthritis HLA
HLA B27 (95% of AS Pts have this; 80% of Reactive arthritis Pts)
onset of Sx in Ankylosing Spondylitis usually occurs before what age
40
Men or women more common in AS
Men 5x more
most common Sx of AS
Uveitis (aka Iritis) = painful red eyes + photophobia, increased lacrimation, blurred vision
complications of AS
- restrictive lung disease
- neurological complications
imaging findings in AS
- bamboo spine (erosion of vertebral bodies)
- Xray of SI joint shows “psuedowidening” of joint d/t sclerosis
imaging to detect early AS
MRI, can see cartilage changes/bone erosions
first line Tx of AS
NSAIDS (Celecoxib)
pharmacologic Tx for AS
- NSAIDs
- DMARDs (Sulfasalazine)
- TNF-alpha agents (Adalimumab)
- Glucocorticoids
Myasthenia Gravis - common age of presentation in women and men
women = 20s yo men = 60s yo
Myasthenia Gravis - autoantibodies created where and against what receptors
created in thymus (85% have thymic hyperplasia) and Abs against acetylcholine receptors (destroy or inhibit them)
Myasthenia Gravis is what kind of hypersensitivity reaction
type 2
most common initial Sx in Myasthenia Gravis
Ptosis (dipoplia can occur from mm weakness)
Sxs in Myasthenia Gravis
- fluctuating mm weakness (worse with exercise, better with rest)
- Ptosis
- Dysphagia
Why is Myasthenia Gravis emergent?
risk of respiratory failure from resp mm weakness
Labs for Myasthenia Gravis
- Edrophonium (Tensilon) test – inhibits AChE, then increased ACh reverses Sxs within 2 mins of injection!)
- Anti-AChE Ab assay (80% sensitivity)
- CT/MRI to screen for thymic hypoplasia/thymoma
- EMG - shows increased jitter
Tx of Myasthenia Gravis
- AChE inhibitors (Donezepil, Rivastigmine) to relieve Sxs
- Immunosuppressive medications (steroids)
- thymectomy
Polyarteritis Nodosa diagnostic criteria
diagnosed if 3 or more are present:
- Weight loss
- myalgia/weakness/leg tenderness
- livedo reticularis (mottled reticular pattern on skin)
- neuropathy
- testicular pain
- diastolic BP elevated > 90
- Elevated Cr or BUN
- HEPATITIS B +
- Arteriographic abnormality (aneurysm)
- Biopsy of artery shows WBCs in artery wall
What is Polyarteritis Nodosa
necrotizing medium vessel vasculitis involving renal, coronary, & mesenteric arteries
5 diseases caused by Necrotizing vasculitis
- polyarteritis nodosa
- RA
- Scleroderma
- SLE
- Wegener’s granulomatosis
Labs in Polymyositis/Dermatomyositis
- serum ANA + sometimes (<30% of cases)
- elevated enzymes (CK from mm, AST, ALT)
- muscle Bx shows lymphocytic infiltrate
- MRI and US may show abnormalities of mm
- CT scan of chest, Abdomen and pelvis may be warranted to screen for malignancy
Physical exams/signs in Polymyositis/Dermatomyositis
- progressive symmetric proximal mm pain and weakness (develops from weeks to months) –> commonly shoulders and hips
- Heliotrope eyelids or “raccoon eyes” (purple-red eyelid discoloration)
- Gottron’s patches (purple papules over knuckles and PIPs)
- Respiratory mm weakness
- Dysphagia/reflux
What MUST the phys exam show in Dermatomyositis
typical dermatomyositis rash (heliotrope eyes, purplish burning cheeks, nose, shoulders, upper chest and elbows)
does polymyositis have a rash
no
what cells cause damage in polymyositis
T cells mediated damage
what cells cause damage in Dermatomyositis
antibody mediated damage
same Sx in polymyositis and dermatomyositis
inflammatory myopathy with symmetrical, proximal muscle weakness
body areas affected by dermatomyositis (not poly)
joints, esophagus, lungs, and rarely the heart
HLA for Rheumatoid arthritis
HLA-DR4
typical age/gender of Rheumatoid arthritis
usually women (3x more than M), 30-50 yo
Sxs/Signs of Rheumatoid arthritis
- morning stiffness > 1 hour, better with use, worse with rest
- symmetric joint involvement of MCPs and PIPs
- B/L ulnar deviation
- Boutonniere deformity (extension of DIP, flexion of PIP)
- Other joints involved (knees, cervical spine, hips, shoulders, elbows)
first choice for imaging in Rheumatoid arthritis-
radiography (plain film Xray)
Disorders with positive RF
- RA, SLE, infections (hepatitis, TB), autoimmune disorders (Sjogrens), and in 1-5% of healthy ppl
Tx for Rheumatoid arthritis
- Swimming
- Pharm:
1. NSAIDs
2. Analgesics (acetaminophin w/ or w/o opioid)
3. Steroids (prednisone)
4. DMARDs (Adalimumab, sulfasalazine)
Sceloderma is what
T cell release of cytokines creates excess collagen synthesis (skin, GI, lungs, kidneys) from T cell release of cytokines
CREST syndrome of Scleroderma
Calcification Raynaud's Esophageal dysmotility Sclerodactyl (claw like fingers) Telangietasias (superficial dilated BV)
Labs for scleroderma
- Antinuclear antibodies - 95% of cases
- Topoisomerase I Abs (aka Scl-70) in 30%
Sjogren is destruction of what
minor salivary glands and lacrimal glands, caused by lymphocytic infiltration
Common Sxs of Sjogrens
xerophthalmia (dry eyes), xerostomia, parotid gland enlargement; dental caries/oral candidiasis/angular chelitis
lab positive in Sjogrens
- serum anti-SSA / Ro (and/or)
- anti-SSB / La
- (or) + RF and ANA
- Parotid Salivary gland biopsy shows lymphoid destruction
Diagnostic criteria of SLE
MD SOAP BRAIN
- Malar butterfly rash
- Discoid rash (immunocomplex deposits along basement membrane)
- Serositis (pleural effusion with friction rub)
- Oral ulcers
- ANA
- Photosensitivity - rash caused by sun
- Blood
- Renal - glomerulonephritis
- Arthritis - osteoperosis
- Immune
- Neurologic - seizures, psychosis, H/A, depression
labs diagnostic in SLE
- Anti-dsDNA and anti-SMITH are SPECIFIC for SLE
other labs in SLE
- ANA
- anti-dsDNA titer and serum complement to monitor Tx
- anti-dsDNA and anti-SMITH to confirm dx
drugs that can cause SLE
- Anticonvuslants (phenytoin)
- AntiHTN (hydralazine)
HLA associated with SLE
HLA B8 and HLA DR
Dose of Epi IM for anaphylaxis
0.3-0.5 mL of 1:1,000 Epi IM to lateral thigh, repeat in 5-15 mins