Ch 17 Systemic Diseases 3 Flashcards
(48 cards)
What are the 2 alternate names for Plummer-Vinson Syndrome?
- Paterson-Kelly Syndrome
2. Sideropenic Dysphagia
What are the 3 components of Plummer-Vinson Syndrome?
- iron-deficiency anemia
- glossitis
- dysphagia
What are the age, gender, and ethnicity demographics for Plummer-Vinson patients?
Females, 30-50 years old, of Scandinavian or Northern European background
What are often found on endoscopy or esophageal barium contrast radiographic studies of Plummer-Vinson patients?
esophageal webs (“abnormal bands” of tissue)
What is the term for the “spoon-shaped” configuration of the nails in some patients with Plummer-Vinson?
koilonychia
What is the diagnostic readout of the blood test for Plummer-Vinson patients?
hypochromic microcytic anemia, consistent with iron-deficiency anemia
(CBC= low MCV, low iron, low ferritin, increased iron binding potential)
Aren’t the numbers SO helpful? What is the % range for the prevalence of upper aerodigestive tract malignancy for Plummer-Vinson patients?
5-50%, a nice, broad range
What age group and ethnicity are affected most often by pernicious anemia?
older patients of Northern European heritage
What is the blood lab diagnosis for pernicious anemia?
megaloblastic anemia: increased MCV, decreased Hg, decreased B12, possibly decreased platelets and WBCs
What vitamin deficiency causes pernicious anemia? What is produced by the parietal cells of the stomach that is needed to absorb this vitamin?
Vit B12, cobalamin (extrinsic factor)…parietal cells make intrinsic factor to absorb B12
WHY is intrinsic factor not being made in pernicious anemia patients? What antibodies are found in these patients?
autoimmune destruction of parietal cells in the stomach, antibodies against intrinsic factor
Besides an autoimmune cause for pernicious anemia, what are 2 other common etiologies that need to be considered?
- s/p gastric bypass surgery 2. vegetarians/vegans
Since cobalamin is essential for nucleic acid synthesis, what two cell types are most often affected in pernicious anemia?
- hematopoietic cells 2. GI lining epithelial cells
What causes the nervous system symptoms (parestheia, numbness, tingling of the extremeties) in pernicous anemia patients?
cobalamin maintains myelin
What are the 2 oral manifestations of pernicious anemia?
- burning sensation (tongue, lips, buccal mucosa)
2. focal patchy oral erythemia/atrophy (tongue affected in 50-60% of patients)
What can Pernicious Anemia reseble histologically?
dysplasia (although the nuclei in pernicious anemia typically are pale staining and show peripheral chromatin clumping)
What are the 3 blood lab markers for pernicious anemia?
- macrocytic anemia
- reduced serum cobalamin
- serum antibodies against intrinsic factor (“quite specific”)
What is the now obsolete test comparing absorption and excretion rates of cobalamin for pernicious anemia?
the Schilling test
How quickly can the oral lesions of pernicious anemia dissipate following intramuscular injections of cyanocobalamin?
5 days
What type of cancer are pernicious anemia patients susceptible to?
gastric carcinoma (7x more likely)
What two deficiencies BOTH cause a megaloblastic anemia?
- Vit B12
2. Vit B9 Folate
What happens if pernicious anemia (Vit B12/cobalamin def) is treated with folate (Vit B9)?
resolution of the anemia and oral lesions, but the reduced myelin production continues resulting in CNS damage
What hormone is pituitary dwarfism related to? What are the two processing centers involved with this hormone?
Growth hormone - hypothalamus releases growth hormone releasing hormone and then GH is released from the Anterior Pituitary
What might be the reason for dwarfism in a patient that has normal or elevated growth hormone?
An AR trait resulting in abnormal/reduced GH receptors in the target cells (there is no treatment, unlike being able to supplement with GHRH or GH)
