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Boards Final Review > CNS + WBC + vascular > Flashcards

CNS + WBC + vascular Flashcards

1
Q

What is the cause of spina bifida?

A

Failure of posterior vertebral arch to close

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2
Q

CNS presentation of a pt with polio? What disorder has a similar presentation to polio?

A

Anterior horn destruction leading to LMN lesion ONLY! [CNS symptoms follow general GI illness as the virus is transferred fecal oral]

[Werdnig Hoffman disease – inherited degeneration of anterior motor horns (autosomal recessive) presenting as a “floppy baby”]

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3
Q

Amyotrophic lateral sclerosis?

A

Degenerative disorder of UPPER AND LOWER MOTOR NEURONS (corticospinal tract). [note that this is different from syringomyelia as there is no loss of pain and temp!]

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4
Q

What is the role of the frataxin gene (mutated in Friedreich ataxia)?

A

Frataxin gene is essential for mitochondrial iron regulation and without that regulation there is increased risk of free radical development and damage of the neurons.

[note that along with cerebellum and spinal cord symptoms there is also an association with hypertrophic cardiomyopathy]

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5
Q

What are the leptomeninges?

A

Pia + arachnoid

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6
Q

Where does the needle stop during a lumbar puncture?

A

Within the arachnoid – it does not piece the pia

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7
Q

What are complications of healing from bacterial meningitis?

A

Hydrocephalus, hearing loss and seizures [this is because of the scarring and fibrosis]

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8
Q

What are the major etiologies of global cerebral ischemia?

A
  1. low perfusion (ex. atherosclerosis)
  2. Acute decrease in blood flow (ex. cardiogenic shock)
  3. chronic hypoxia (ex. anemia)
  4. Hypoglycemia - glucose is essential energy source, so repeated episodes diffusely affect the brain
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9
Q

What is the general result of MODERATE global ischemia?

A

causes infarcts in watershed areas and damage to highly vulnerable regions…

  1. pyramidal neurons of cerebral cortex (layers 3,5,6) [creating cortical LAMINAR NECROSIS]
  2. pyramidal neurons of the hippocampus (temporal lobe)
  3. Purkinje layer of the cerebellum - what integrates sensory perception with motor control
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10
Q

What type of infarct is caused by thrombotic vs embolic stroke?

A

Thrombotic - pale infarct [thrombus cannot be lysed b/c you still have the subendothelial collagen present due to atherosclerotic plaque rupture]

Embolic - red infarct [b/c you occlude vessel, then it is lysed allowing blood to rush back in]

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11
Q

What layer is lacking in berry aneurysms making it susceptible to formation and rupture?

A

Media layer lacks

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12
Q

Lens shaped hematoma? Crescent shaped hematoma?

A

Lens shaped hematoma - EPIDURAL hematoma

Crescent shaped hematoma - SUBDURAL hematoma

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13
Q

What are the 3 complications of an uncal herniation?

A
  1. compression of CN III (eye moves “down and out”)
  2. compression on PCA leading to occipital lobe infarct
  3. rupture of paramedian artery leading to DURET (BRAINSTEM) HEMORRHAGES
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14
Q

What is metachromatic leukodystrophy?

A

Deficiency of arylsulfatase (autosomal recessive) preventing degradation of sulfatides and therefore accumulate in the lysosomes of oligodendrocytes

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15
Q

What is Adrenoleukodystrophy?

A

Impaired addition of coenzyme A to long-chain fatty acids (X-linked defect) – the accumulation of fatty acid damages adrenal glands and white matter of the brain

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16
Q

What is the characteristic histology of Pick’s disease?

A

Degeneration of frontal and temporal cortex (sparing occipital and parietal lobes) with ROUND aggregates of TAU PROTEIN (pick bodies) in the cortex

17
Q

What type of rosettes are found in medulloblastoma and ependymoma?

A

Medulloblastoma - homer-wright rosettes

Ependymoma - perivascular pseudorosettes

18
Q

What is the most sensitive cell in the body to radiation?

A

Lymphocytes

19
Q

What receptor/cell marker is decreased with immature neutrophils in the circulation?

A

Decreased Fc receptor (CD16)

20
Q

What are the major causes of increased eosinophils in the blood?

A
  1. allergic reaction (type I hypersensitivity)
  2. parasitic infections
  3. Hodgkin’s lymphoma (due to elevated IL-5)
21
Q

What disorder is associated with basophilia?

A

CML - elevated granulocyte in blood smear

22
Q

Why is there elevated lymphocytes in Bordetella pertussis infections?

A

Bacteria releases lymphocytosis-promoting factor which blocks circulating lymphocytes from leaving the blood to enter the lymph node

23
Q

What is the hallmark marker of ALL?

A

tDt+ in the nucleus [DNA polymerase present ONLY int eh nucleus of lymphoblasts]

24
Q

What blood disorders are associated with Down Syndrome?

A

AML - before the age of 5

ALL - after the age of 5

25
Q

Where do pts who have B-ALL treated with chemotherapy need prophylaxis to?

A

scrotum and CSF prophylaxis

26
Q

What are the chromosomal changes seen with B-ALL?

A

t(12;21) – good prognosis,more common in children

t(9;22) – poor prognosis, more common in adults (Philadelphia chromosome positive)

27
Q

Which subset of AML is associated with infiltration of the gums?

A

Acute MONOCYTIC leukemia

28
Q

What stain is used for hairy cell leukemia?

A

TRAP stain

[also note that these pts have splenomegaly due to red pulp, there is dry bone marrow tap and no lymphadenopathy]

29
Q

What is the best treatment for hairy cell leukemia?

A

2-CDA (cladribine)

[adenosine deaminase inhibitor allowing adenosine to accumulate to toxic levels in the neoplastic B cells]

30
Q

lytic bone lesions with hypercalcemia AND rash?

A

adult T-cell leukemia/lymphoma

[neoplastic proliferation of mature CD4+ T cells associated with HTLV-1]

31
Q

What is a Pautrier microabscess? Sezary syndrome?

A

Pautrier microabscess - Aggregate of mature CD4+ neoplastic cells in the epidermis associated with mycosis fungoides

Sezary syndrome - mycosis fungoides involving the blood with characteristic lymphocytes with cerebriform nuclei (sezary cells) [cerebriform b/c lobe appearance resembles the brain]

32
Q

What are complications of myeloproliferative disorder?

A
  1. increased risk for hyperuricemia and gout (DOES NOT occur with essential thrombocythemia b/c platelets are just pinched off cytoplasm from larger megakaryocytes]
  2. progression to marrow fibrosis
  3. transformation to acute leukemia [with CML it can progress to AML (2/3) or ALL (1/3) b/c the mutation is in the pluripotent stem cell]
33
Q

What are the major difference b/t a leukemoid reaction and CML?

A
  1. negative leukocyte alkaline phosphatase (LAP) stain in CML [positive in leukemoid reaction]
  2. increased basophils in CML [no increase with leukemoid reaction]
  3. t(9;22) present in CML [no translocation in leukemoid reaction]
34
Q

Mantle Cell lymphoma?

A

Neoplastic proliferation of small B cells (CD20+) that expand the region immediately adjacent to the follicle - driven by a t(11;14) translocation moving cyclin D1 on chromosome 11 to Ig heavy chain so overexpression of cyclin D1 promotes G1/S transition in cell cycle – presents in late adulthood with painless lymphadenopathy

35
Q

What is the most common form of non-hodgkin’s lymphoma?

A

Diffuse large B-cell lymphoma

36
Q

Which subset of patients generally present with lymphocyte-depleted hodgkin’s lymphoma?

A

Elderly and HIV-positive pts – this is the most aggressive, worst prognosis associated with hodgkin’s lymphonma

37
Q

What is the cause of lytic bone lesions in multiple myeloma?

A

Elevated serum IL-6 stimulating plasma cell growth and Ig production - Neoplastic plasma cells then activate the RANK receptor on osteoclasts leading to bone destruction [“punched out” lesions] – increasing risk of fracture and levels of calcium due to breakdown of bone and release of calcium

38
Q

What is langerhans cell histiocytosis?

A

Neoplastic proliferation of specialized dendritic cells found predominantly in the skin – they are derived from bone marrow monocytes and are CD1a+ and S100+ – Birbeck granules

3 subtypes…

  1. Letterer-Siwe disease - malignant, rash, infants less than 2yo
  2. Eosinophilic granuloma - benign, pathologic fracture (bone), no skin involvement, langerhan cells with mixed inflammatory cells esp eosinophilis
  3. Hand-schuller-christian disease - malignant, skin invovlement, diabetes insipidus, exopthalmos and present in children over the age of 3

Boards Final Review (7 decks)

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