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110 Cards in this Set

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what is an absolute reduction in the number of granulocytes in the blood to below 1000/mm3
granulocytopenia (primarily a neutropenia)
what 3 drugs are associated with drug-induced cessation of granulopoiesis
aminopyrine, sulfonamides, and chloramphenicols
what drug results in immune mediated cytolysis or leukoagglutination
aminopyrine
what is a replacement of hematopoietic tissue in the bone marrow by abnormal tissue, usually fibrous tissue or malignant tumors which are most commonly metastatic carcinomas
myelophthisis
what is an intrinsic neutrophil defect which allows normal proliferation of bone marrow precursor cells, but results in abnormal granulogenesis and inability to form secondary granules.
familial neutropenia
children affected with familial neutropenia develop what and what is their blood neutrophil count
persistant bacterial infections. count is less than 1000/mm3
what is an autosomal dominant condition in which no bone marrow granulocytes are produced beyond the myelocyte stage because of cyclic interruption of the production of the pluripotent bone marrow stem cells. the cycle occurs every 21-28 days, and the 4-10 day neutropenia results in illness, malaise, sore throat, gingival ulcers, and skin abscesses
cyclic neutropenia
aplastic anemia results in what
suppression of myeloid stem cells
what condition causes intrasplenic destruction or sequestration of neutrophilic leukocytes, and is also observed with rheumatoid arthritis and antineutrophil antibody production
Felty's syndrome (hypersplenism)
vitamin b12 and folate deficiencies result in what
in ineffective granulopoiesis
what condition results in antibodies produced against cell surface antigens that can result in cytolysis and or leukoagglutination. usually associated with aplastic and hemolytic anemia, and thrombocytopenia.
idiopathic autoimmune neutropenia
what neutropenia is due to transplacental transfer of IgG immunoglobulin produced by the mother against granulocyte surface antigens of her infant, and granulocyte survival is shortened
isoimmune neutropenia
what can be used to improve prognosis for granulocytopenia
antibiotic therapy and or recombinant human granulopoietic factors
what condition shows neutrophils that phagocytize normally but do not destroy the ingested pathogen. the defect is an absence or inactivity of the membrane-associated NADPH oxidase enzyme that generates superoxide anions. often seen in children. is autosomal recessive. and patient suffereing with the condition have recurrent infections caused by staph and gram - rods.
chronic granulomatous disease
what condition is a deficiency that lies with the lack of NADPH production by the hexose monophosphate shunt, and in children an increase in susceitibility to infection is seen due to the inability of neutrophils to generate the respiratory burst
glucose 6 phosphate deficiency
what condition in children is an autosomal recessive inheritance. it involves a delay in fusion of abnormal azurophil granules with phagolysosomes, and is characterized by dilution in color of the hair and eyes, a platelet storage defect, and an increased susceptibility to bacterial and viral diseases
chediak-higashi syndrome
what condition is a benign anomaly of leukocytes inherited as an autosomal dominant inheritance, with a reduced number of nuclear segments, best seen in neutrophils, where the incompletely segmented nuclei appear round or oval
pelger-huet anomaly
what results from a neutrophil mobility difficulty with resultant infection with capnocytophaga, an anaerobic bacillus
juvenile periodontitis
what condition is a failure of B-cell precursors to differentiate into mature B cells, because of a mutation in cytoplasmic tyrosine kinase. it shows a marked depression of all serum immunoglobulins, is x-linked inheritance, and shows recurrent infections with pyogenic bacteria when maternal immunoglobulins are lost.
x-linked agammaglobulinemia of Bruton
what condition shows a lack of germinal center in the lymph nodes, tonsils, appendix, and spleen, and the gut lacks peyers patches. also plasma cells are absent and B cell compoenent is nearly absent. this condition often develops autoimmune CT disorders
x-linked agammaglobulinemia of Bruton
what is used to diagnose and treat x-linked agammaglobulinemia of Bruton
diagnosed with immunoelectrophoresis and treated with gamma globulins and antibiotics
what is a very common selective immunodeficiency disease showing a near absence of serum and secretory IgA, causing a mucosal defensive weakining which results in infections in the gi, respiratory, and urogenital systems. symptoms inclue recurring sinopulmonary infections and diarrhea, and is treated with antibiotics
isolated IgA deficinecy
what form of isolated IgA deficiency results from a basic defect int he terminal differentiation of B lymphocytes to plasma cells
hereditary form
what form of isolated IgA deficiency results from certain viral infections
acquired form
what condition is a selective T-cell deficiency resulting from failure of devt of the 3rd and 4th pharyngeal pouches, the thymus is absent and a total lack of cell-mediated immune response is seen. the peripheral lymphocyte count is decreased and patients suffer from an increased susceptibility to viral and fungal infections. also paracortical areas of the spleen and lymph nodes are depleted, and can be associated with cardiovascular, cerebral, and endocrine defects
DiGeorge syndrome
lack of parathyroids in digeorge syndrome results in what
tetany
what is digeorge syndrome treated with
fetal thymic transplants
what condition is a defect in both T and B cells being deficient in the blood, lymph, and spleen. the thymus gland fails to descend from the neck into the anterior mediastinum and is devoid of lymphoid cells and hassalls corpuscles. IG are not produced and children suffering from this often die before age one due to infections with pseudomonas, candida or viruses
severe combined immunodeficinecy disease
pneumonias from severe combined immunodeficiency diseases are caused by what
pneumocystis carinii
what is used to treat severe combined immunodeficiency diseases
germ-free envt, bone marrow stem cell transplants and or gene therapy
in what condition is there a delay in the maturation of B cells and their immunologic function. the gamma globulin level is much lower than usual for that age of infant, and they suffer from overt infections, fever and bronchitis
transient hypogammaglobulinemia
what condition is due to deficiency in C1 esterase inhibitor, is transmitted as an autosomal dominant trait, and allows complement cascades to continue unchecked. results in recurrent episodes of edema and swelling due to an increased permeability of the vasculature. it is provoked by emotional distress or trauma
hereditary angioneurotic edema
what is the tx for hereditary angioneurotic edema
c1 esterase inhibitor concentrates prepared from human plasma
most malignant lymphomas are of what cell origin
monoclonal B cell origin
what NHL classification type is based on cell types, where cell types and morphology are identified with immunohistochemical cell markers
lukes collins
what type of lymphoma can be of african or american variety, and appears to be associated iwth the epstein barr virus. it is different from other lymphomas because it rarely arises within peripheral lymph nodes, and usually arises from mature B cells in primarily extranodal locations
Burkitt's lymphoma
what NHL classification system is based solely on morphologic criteria, specifically on the distribution of the neoplastic cells within the lymphoma and the cytologic characteristics of the neoplastic cells
rappaport
lymphoid neoplasms tend to disrupt normal immune regulatory mechanisms resulting in what
increased susceptibility to infection and autoimmunity
what type of lymphoma are distinguished by their distinctive morphology (Reed-sternberg cell), origin, and route of spread. more common in men than women. almost alwasy arises within lymph nodes, spreads in predictable fashion from lymph nodes to spleen to liver to bone marrow and then extranodal involvement.
hodgkins lymphoma
what classification system is based primarily on lymphocyte population in comparison to the histiocytes and RS cells
rye classificaiton
what is the most common type of hodgkins disease
nodular sclerosis
describe an Reed-steinberg cell
large, often binucleated or bilobed and contains owl eyed nucleoli
what is a malignant hematopoietic neoplasm characterize by a disordely purposeless proliferation of blood cells or their precursors, where the bone marrow is always involve, and an abnormal number of immature white blood cells are often present int he peripheral blood
leukemia
what type of leukemia is seen in people over 50, it has a subtle onset, a sluggish course often lasting 10 to 15 years and increased number of small lymphocytes. usually involves immunologically defective B cells, and death results from infections and organ infiltration. it is also the most common form of leukemia in adults of the western world
chronic lymphocytic leukemia
what type of leukemia occurs most often in children, has a sudden onset, marrow is packed with lymphoblasts, and causes death quickly if untreated. it is the most common form of cancer in children under 15.
acute lymphoblastic leukemia
what type of leukemia is predominated by the presence of myeloblasts, and is seen in patients 15-39
acute myeloblastic leukemia
what type of leukemia has onset in the 3rd and 4th decade of life, features splenomegaly without lymphadenopathy, and leukocytosis due to increased numbers of fairly mature granulocytes. also has chromosomal aberrations, especially the philadelphia chromosome
chronic myelocytic leukemia
what type of leukemia includes plasma cell tumors and multiple myeloma. results in gammopathies and punched out bone lesions
plasmacytic leukemia
splenomegaly is most common with what type of leukemia
CML
what is the most common developmental abnormality of the esophagus
atresia or fistula
what is the most common collagen disease affecting the esophagus
scleroderma
what are annular narrowings of the upper esophagus, often associaed iwth iron deficiency in women over 40.
esophageal webs and rings
the combination of iron deficiency anemia, atrophic glossitis, cheilosis, and dysphage is known as what
plummer-vinson or patterson-brown-kelly sydrome
what is a disorder of esophageal motility resulting from a failure of the gastroesophageal sphincter to relax and loss of normal peristalsis of the distal two thirds of the esophagus due mostly to loss of myenteric ganglion cells. leads to progressive dysphagia, regurgitation of ingested food and possibly aspiration of ingesta with resulting aspiration pneumoniae
achalasia
what is a disorder of the gastroesophageal junction, resulting in a saclike dilation of the stomach protruding above the diaphragm.
hiatal hernia
the reflux of gastric contents into the lower esophagus often induces what
GERD
what is an abnormal outpouching of the esophageal mucosa and submucosa through a weakness in the muscular wall usually due to an increased intraluminal pressure or eversion of all layers of the esophagus resulting from healing of an inflammatory process external to the esophagus
diverticula
what condition is characterized by linear longitudinal lacerations of the mucosa and submucosa involving the distal esophagus most often seen after prolonged vomiting, especially in chronic alcoholics
mallory-weiss syndrome
what cells secrete mucus and pepsinogen I and II
mucous cells
what cells secrete acid and intrinsic factor which allows absorption of vitamin B12 in the ileum)
parietal cells
what cells secrete the proteolytic pepsinogen I and II
chief cells
what cells are gastrin secreting endocrine cells
G cells
what do enteroendocrine cells release
histamines
what is a weakness or partial to total absence of a region of the diaphragm which allows the abdominal contents to herniate into the thorax. this may lead to vomiting or burping
diaphragmatic hernias
what is a narrowing of the pyloric lumen
pyloric stenosis
what condition is an acute mucosal inflammation that is usually transient. can be caused by smoking, aspirin, alcohol, and drugs. can vary from mild neutrophil infiltration to diffuse sloughing of the gastric mucosa with hemorrhage. can be associated with epigastric pain, nausea, and vamiting along with hematemesis.
acute gastritis
what condition is often idiopathic but can be associated iwth pernicious anemia. patients produce autoantibodies directed against intrinsic factor and gastric parietal cells. the pt develop vitamin b12 deficiency anemia, atrophy of gastric glands with resulting hypo or achlorhydria and loss of parietal cells.
chronic gastritis
what lesions represent extension of the acute erosive gastritis through the muscularis mucosa to become an ulcer. they are associated with extensive burns, acute brain damage, surgery, shock, asprin abuse, steroids, and smoking
acute gastric ulceration
what lesions are usually solitary lesions resulting from acid digestion of the mucosa and occur in the duodenum and stomac (4:1 ratio). not common before 20 yoa and more common in men than women
chronic gastric ulcers
which appear first: gastric ulcers or duodenal ulcers
duodenal ulcers
what condition is often associated with zollinger-ellison syndrome (in which individuals contain a gastrin producing tumor of the pancreatic islets)resulting hypergastrinemia and hypercholrhydria leads to gastric mucosal hyperplasia and hypertrophy and multiple gastric ulcers. gastric rugae become greatly thickened.
chronic hypertrophic gastropathy
what congenital anomalie of the small intestine causes intestinal obstruction that leads to persistant vomiting, usually within the first wo weeks of life, it results in failure of one or more segments of the small bowel to develop a lumen
atresia and stenosis
what is a herniation of the mucosa and submucosa of the small intestine through the muscle wall where the mesenteric vessels and nerves enter and provide a focus of weakness
multiple diverticula
what is a persistence of the omphalomesenteric duct remnant. located about 12 inches from the ileocecal valve. can contain heterotropic nests of gastric mucosa and can develop peptic ulcers and bleed. when these become infected and or inflamed they will mimic acute apendicitis
meckels diverticulum
what is an occlusion of either the arterial supply or the venous drainage of the small bowel, mostly related to emboli from vegetative valvular endocarditis or atherosclerosis of mesenteric vasculature. can result in transmural hemorrhagic infarction. bowel gangrene and perforation with resulting death are potential outcomes
mesenterich thrombosis
what is a non thrombotic intestinal infarction due to hypoperfusion of the gut wall following shock, cardiac failure, or vasoactive drug administration. can result in hemorrhagic mucosal or mural infarction. usually the serosa and subserosal tissues are spared
acute hemorrhagic enteropathy
what is a complete twisting of a loop of bowel about its mesenteric base of attachment leading to intestinal obstruction and infarction
volvulus
what condition is due to a failure of the meissners and auerbachs plexus to develop. occurs at the anorectal junction and extends proximally to a variable extent but usually is limited to the rectum or sigmoid colon. the loack of peristalsis creates a functional obstruction which results in dilation of the proximal bowel. is ten times more frequent in downs patients and four times more frequent in males
congenital aganglionic megacolon (Hirschsprugs disease)
what condition is a transmural, chronic relapsing granulomatous inflammatory disease which may affect any portion of the GI tract, especially the ileum and colon. caused predominantly by autoimmunity. gross morphologic lesions have a segmented arrangement (skip lesions) and include transmural fibrosis and thickening of the bowel wall, narrowing of the bowel lumen, and serpentine ulceration of the mucosal surface, fissures, and fistulas. characterized microscopically by noncaseating granuloma formation
crohn disease
what is the most worrisome polyp
villous adenoma greater than 4 cm
what is any tumorous mass that protrudes above the surface of the srrounding mucous membrane originating from mucosa or submucosa.
polyp
an inflammatory bowel disease of unknown etiology that oriniates in the rectus, spreads proximally, and it is characterized by mucosal ulceration, crypt, abscesses, and the absence of skip lesions
ulcerative colitis
the trachea is lined by what type of cell
pseudostratified ciliated columnar epithelium
the lower airway epithelium consist of what three primary cell types
ciliated, reserve cells, and goblet cells
what is ther terminal respiratory unit that includes all structures beyond the terminal bronchiole, such as the respiratory bronchiole, alveolar duct, alveolar sac, with the corresponding circulation
acinus
what is the pyramidal structure bordered on its sides by a discontinuous layer of pulmonary septum and is comprised of three to five acini
primary lobule
the cytosol of type I pneumocytes can extend up to 50 microns away from the nucleus and into what structure which connects alveoli of different lobules
pores of Kohn
what are the reserve cells of the alveolus that are also responsible for the production of surfactant
type II pneumocytes
what is the term for either an incomplete expansion of the lungs or to a collapse of previously inflated lungs, primarily involving the alveoli. most of these are reversibl
atelectasis
what condition is caused by severe diffuse alveolar capillary membrane injury related to numerous potential infectious and non-infectious etiologies. best characterized by edema, fibrin exudation, and hyaline membrane formation within the alveolar sacs, and type II pneumocyte proliferation. It often becomes chronic resulting in fibrosis and scarring with permanent loss of lung compliance
adult respiratory distress syndrome
what condition most common in women from 20-40 is involved with BMPR2 (morphogenic protein receptor 2) where the artery walls become thickened due to medial hypertrophy, elastic thickening of the intima, duplication of the interal elastic membrane, and plexogenic lesion formation.
pulmonary hypertension and vascular sclerosis
what group of conditions is characterized by dyspnea and results from the narrowing or obstruction of the trachebronchial tree or from destruction of the lung parenchyma.
obstructive pulmonary disease
what condition is an abnormal permanent enlargement of the air spaces distal to the terminal bronchiole, accompanied by destruction of their walls, without obvious fibrosis. it involves a protease-antiprotease mechanism, whereby deposition of particles or smoke at the terminal bronchioles induces an inflammatory reaction. Ultimate result is from the destructive effect of high protease activity in people with a low antiprotease activity. symptoms do not appear until at least 1/3 of the functional lung parenchyma is incapacitated, and the major disruption is the distal airway tissue destruction with resultant loss of elastic recoil rather than the large airway obstruction observed with chronic bronchitis
emphysema
what type of emphysema involves the respiratory bronchioles (central or proximal portion of the acinus) and spares the distal alveoli. it occurs most commonly in heavy smokers and is observed along with chronic bronchitis. represents greater than 95% of all emphysema cases
centriacinar or centrilobular emphysema
what type of emphysema involves all areas of the acinus. it occurs mostly in people with autosomal recessive genetically determined alpha-1-antitrypsin deficiency and is enhanced by smoking
panacinar (panlobular) emphysema
what type of emphysema involves only the distal portion of the acinus and spares the respiratory bronchiole. it may lead to spontaneous pneumothorax, especially in young adults
paraseptal (distal acinar) emphysema
what type of emphysema is often associated with scarring and has minimal clinical significance. it is often present in older adults and occurs within old scards due to previous infections
irregular
what condition is characerized by pts who have a persistant cough with sputum production for at least three months in at least two consecutive years with no other recognized cause. associated with chronic irritation by inhaled substanced and is exacerbated by microbiologic infections. increased mucus secretion, stasis of cilia, and decreased clearance of substances from the lungs are often clinical features.
chronic bronchitis
what condition involves a chronic relapsing inflammatory condition associated with widespread narrowing of the bronchial airways which changes in severity over short periods of time. occurs in 5% of children, is reversible, responds to bronchodilaters, with elevated serum IgE levels. coughing and wheezing usually result from the excess mucus production and bronchoconstriction
bronchial asthma
what condition is an abnormal permanent dilation of bronchi and bronchioles resulting from inflammtory damage to their walls. it is often associated with airway obstruction due to a chronic necrotizing infection. involves coughing up copious amounts of purulent sputum and is most commly found in those hereditary diseases that produce ciliary dysfunction
bronchiectasis
what condition results from impaired defense mechanisms of the lung, usually seen in infants and the elderly, and caused by many different bacteria.
pneumoniae
what type of pneumonia is characteized by a patchy consolidiation
bronchopneumonia
what type of pneumonia involves a large portion or an entire lung lobe.
lobar pneumoniae
what are the best two examples of chronic granulomatous pneumonia
pulmonary tuberculosis and fungal infections
what group of disorders is characterized by diffuse and usually chronic involvement of the pulmonary CT, especially the interstitium in the alveolar walls (alveolitis) resulting in loss of lung capacity. can lead to severe chronic interstitial fibrosis. causes include pneumoconiosis, hypersensitivity pneumonitis, diffuse interstitial fibrosis, and granulomatous diseases. similar pathogenesisi to ARDS but more directed at type I pneumocyte damage and resulting fibrosis. ends in honecomb lung characterized by numerous cystic spaces seperated by dense fibrous scars and often filled with mucus and cellular debris
diffuse interstitial diseases
what lung neoplasm makes up 95% of all lung neoplasms. it is the most common lethal visceral malignancy in both females and males. related to smoking, industrial hazards, and air pollution as well as genetic factors. can be of squamous cell, adenocarcinomic, or small cell or large cell carcinomic types
bronchogenic carcinoma
what type of bronchiogenic carcinoma is most often seen in smokers and which of these two has the poorest prognosis
squamous cell and small cell carcinomas are most frequently seen in smokers and small cell carcinoma has the poorest prognosis
what type of neoplasm represents a variant of the adenocarcinoma and usually arises from the alveolar lining cells or from terminal bronchioles
bronchioalveolar carcinoma
what type of neoplasm of the lungs arises from argentaffin (kulchitsky) cells
bronchial carcinoid
what is the most common site for metastatic cancers
the lungs