Haematology PATH Flashcards
Hodgkin vs Non-Hodgkin lymphoma
Hodgkin
- Single group of nodes
- Spread by contiguity
- Mesenteric nodes and Waldeyers ring rarely involved
- Extranodal involvement uncommon
- Reed-Sternberg cells
- CD30 (no CD 20)
NHL
- Multiple group of nodes
- Non-contiguous spread
- Mesenteric nodes and Waldeyers ring involved
- Extranodal invovlement
- No RS cells
- CD20
Hodgkin Lymphoma
- Epi
40% of lymphomas.
Bimodal age peak at 25 and 75. Overall slight male predominance.
4 types of Hodgkin lymphoma
- Nodular sclerosis (70%)
- common in women
- excellent prognosis - Mixed cellularity (20%)
- EBV associated - Lymphocyte-Rich (uncommon)
- Good prognosis - Lymphocyte poor
- WORST prognosis
- EBV positive* - Lymphocyte preDOMINANCE
- BEST prognosis
- Popcorn cells
Hodgkin lymphoma risk factor ?
- EBV
- Immunosuppression
- Connective tissue disorders
- RA, SLE, Sarcoid, UC
Morphology of Hodgkin Lymphoma
- RS cells in a background of non-neoplastic inflammatory cells **
Hodgkin lymphoma prognosis
- Curable in most cases*
How does PTH increase serum calcium ?
- Kidney
- increase renal absoprtion
- increase conversion of vitamin D to active form - Increase GI absoprtion
- Increase RANK-L expression to increase osteoclast function
Most common cause of hyperparathyroidism
- The most common cause of primary hyperparathyroidism is a sporadic parathyroid adenoma
- Familial syndromes are a distant second
How to make the diagnosis of Multiple Myeloma
- Monocloncal immunoglobulin
- Free Light chains
- Bence-Jones proteins (directly nephrotoxic)
- Tamm Horsfall proteins
- Get amyloidosis AL type in kidney*
- Rouleaux formation (high M chains)
However, 1% are non-secretors with nothing detectable in blood or urine
What’s the major pathological feature of Multiple Myeloma ?
Unregulated expression of RANKL, which activates osteoclasts.
Multiple myeloma
- associations
Urate neprhopathy
Amyloidosis
POEMS - an atypical form of myeloma – polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes
Follicular NHL features and prognosis
- Indolent but incurable*
- 40% transforms to DLBCL
Marginal zone lymphoma (NHL) prognosis
Can regress if inciting stimulus removed. Hallmark is lymphoepithelial lesion
Burkitt lymphoma association
- EBV, HIV
Burkitt morphology
Fish flesh tumorous masses. Also a term/appearance sometimes for sarcomas.
Distribution: 60% nodal vs 40% extranodal (spleen usually)
What cells do you find in Parathroid hyperplasia ?
” Chief cell “ Hyperplasia
B-thalassemia or Alpha more common ? Which one more severe usually
B-Thalassemia more common and more severe usually
3 alpha gene deletion and 4 alpha gene deletion is called ?
3 alpha gene deletion = HbH (clinically resembles B-thalassemia intermedia)
4 alpha gene deletion = Hb Barts (Long term survival now possible with early recognition, intrauterine transfusion, stem cell transplant)
NF-1 or NF-2 more common?
NF-1 by far
NF-1 which chromosome mutation and gene product ?
Loss of Chr 17 -> decreased “neurofibromin”
Plexiform Neurofibroma complication ?
- A plexiform neurofibroma has a lifetime risk of up to 10% of transformation into a malignant tumour
Main cause of death for NF-1 ?
Malignant peripheral nerve sheath tumor
NF-2 epidemiology
- Occurs in 1 in 40-50,000.
- Rarely noticed prior to puberty
- Mean age of diagnosis ~25 years
NF-2 loss of gene on what chromosome
- Loss of NF2 gene = chr22q12.
- NF2 functions as a tumour suppressor gene (encodes for Merlin protein).
NF-2 prognosis
Poor prognosis, mean age of death 36
Resection of CN8 schwannomas with hearing preservation is possible
Genetic counselling
TS inheritance ?
- Sporadic most common (60%)
- Minority familial: AD - can skip generation due to low expressitivity
VHL clinical presentation
Visual loss from retinal haemangioblastoma
VHL complication
Renal cell carcinoma is a major cause of morbidity and mortality – 70% risk by age 60.
GRANULOMATOSIS with POLYANGIITIS (Wegners)
- Pathophysiology ?
- C-ANCA positive in how many %?
Probably a T-cell mediated hypersensitivity response to normally “innocuous” inhaled microbial or other
environmental agents (Robbins).
C-ANCA positive in up to 95% of cases.
Granulomatosis with Polyangiitis
- most common location ?
Lungs > paranasal sinuses > kidneys > joints > eye > skin
Indications for AAA surgery
- ascending aorta >5.5 cm
- descending aorta >6.5 cm
- growth rate >1cm/year
- symptomatic patient
Mycotic aneurysm cause ?
- Embolisation of septic embolus (usually from infective endocarditis)
- Extension of adjacent suppurative process or circulating organisms directly infect the arterial wall.
Inflammatory aneurysm shape ?
- Fusiform
Adult form Coarctation of Aorta presentation ?
- Presents later, often well into adulthood
- Upper extremity hypertension with weak pulses and hypotension in the lower extremities
- Manifestations of arterial insufficiency (ie claudication, cold legs).
- Murmurs
What vessels causes rib notching in Aortic Coarctation ?
- intercostal and internal mammary arteries
Aortic coarctation associations
- Bisucpis aortic valve (50%)
- VSD, ASD, mitral regurg, berry aneurysms, aortic stenosis
- Turners
Infantile vs Adult form Aortic coarctation
Infantile
- Preductal
Adult
- Postductal
Stanford A more common or B?
- A more common
RF of aortic dissection
- HTN (90%)
- Aortic Coarctation
- Bicuspid aortic valve
- Connective tissue disorders
- Cardiac catheterisation
Morphology seen in Dissection and ANeurysm
- Cystic medial degeneration
- Intimal tear into but not beyond the media – the blood filled false lumen is usually in a plane between the middle and outer 1/3 of the media.
Do all aortic dissection have intimal tears ?
No, 5-10% dont have obvious intimal tears
Are all aortic dissection detectable on Radiology ?
- No
Atheroma development pathophysio first step
Endothelial injury (vascular permeability, leukocyte adhesion)
Atheroma composition
- Lipid core with fibrous cap
- Periphery (neovascularisation).
What are Fatty streaks
- Early lesions made of lipid laden foamy macrophages in the intima that only mildly protrude into the lumen
- Occur at any age even in kids and not all go on to become atheromatous plaques, although some do
T-cell lymphoma high or low grade ?
T cell = all high grade
Primary vs Secondary Syphilis features
Primary syphilis: painless chancre with nontender lymphadenopathy
Secondary: Papulosquamous, Rash skin lesions
Tertiary: gummatous lesions, neurologic or cardiovascular symptoms
Extra-adrenal paraganglioma most common site ?
Approximately 85% arise below the diaphragm
Paraganglioma more commonly maglignant if ..?
- Extra-adrenal
- Related to syndrome
Most common parasympathetic paraganglioma ?
- Carotid body paraganglioma
Hashimoto labs ?
- Lymphocytic aggregates
- Transformed follicular cells (Askanazy/oxyphilic/Hurthle cells)
- Anti-thyroglobulin antibodies (70%)
- TPO (95%)
- TSH (High)
- Free T4 (Low)
Hashimoto complication
- Non-hodgkin lymphoma (majority)
- Papillary thyroid cancer (1.7x increased risk)
- Medullary thyroid cancer (2.7x increased risk)
