Robbins Essential Pathology p137-142 PDF

112 CHAPTER 7 Diseases of Blood Vessels  erapy, and surg c a  rep a r o  e aor   c  n  ma  e ar c an s ave 65% Pathogeness. he carac...

112 CHAPTER 7 Diseases of Blood Vessels  erapy, and surg c a  rep a r o  e aor   c  n  ma  e ar c an s ave 65% Pathogeness. he caracersc granuomaous nlammaon, an asso- o 85% o  e p a ens w   d ss e c   on  nvov  ng  e aor   c arc. Ds - caon w ceran HLA cass II apoypes, and e exceen era- s e c  ons  a do no  nvove  e aor   c arc c an be re p a re d surg - peuc response o serods a suppor an mmune eoogy. Gan ce c a  y or manage d c ons er va vey w   an  y p e r ens ve d r ugs and arers key occurs as a resu o a cronc h1 ce–medaed mmune ave s ome w a b eer oucomes. response, presumaby o angens presen n e vesse wa. he deny o ese angens and e bass or e predecon or vesses o e ead are unknown. VASCULITIS Vasculitis encompasses a group of disorders with highly varied Morphology. Granu omaous nl amma on o cc urs n a p acy pathophysiology and clinical features that are dened by the pres ds r bu on a ong  e eng  o afe c e d vess es. Invove d ar er  a  ence of vessel wall inammation. s eg mens exb no du  ar n ma   ckenng (and o cc asona  Cnca manesaons depend on e specc vascuar bed a  romb os es)  a mpnge on  e umen and c aus e ds a  s cem a. s afeced and somemes ncude sgns and sympoms o sysemc Fu  y de veop e d esons conss o nonne cro zng g ranu omas nlammaon. Many orms o vascus are recognzed, some w w   mu  nuce ae d g  an ce s cenere d on  e ner na  e as c overappng eaures. Eoog y s dverse and ncudes mmune-me- membrane, w c s oten  rag mene d (Fg. 7.10). He a ng o daed nlammaon, necons, drugs and cemcas, radaon  e njur y s ass o c ae d w   n ma   ckenng ,  nnng and exposure, and rauma. I s crca o dsngus necous rom s c ar r ng o  e me d a, and adven   a  bross. mmune-medaed orms because mmunosuppressve erapy s approprae or e aer bu coud exacerbae necous vascus. We w resrc our dscusson o orms o vascus a are mos com- Clncal Features. Gan ce arers s e mos common orm o vas- mon or o greaes paogenc neres, sarng w ose a prmar- cus among oder adus n ger-ncome counres. I s rare beore y afec arge vesses. 50 years o age. Sgns and sympoms may relec sysemc nlamma- on (ever, maase, weg oss) or ake e orm o aca pan or Giant Cell (Temporal) Arteritis eadace, mos nense aong e empora arer y, wc s panu o Giant cell (temporal) arteritis is a chronic granulomatous disorder papaon. Ocuar sympoms appear abrupy n abou 50% o paens that principally affects large-sized arteries in the head. and range rom dpopa o compee vson oss. Dagnoss requres he empora, verebra, and opamc ar eres and e aora can bopsy, ypcay o e empora arer y ; owever, because e arers be nvoved. Opamc ar er y nvovemen can cause sudden and s pacy, a negave bopsy resu does no excude e dagnoss. Cor- permanen bndness; ereore, promp dagnoss and reamen are coserod or an–umor necross acor (TNF) erapes are efecve essena. reamens. A B Fig. 7.10 Giant cell arteritis. (A) Hematoxylin-and-eosin (H&E)–stained section of a temporal artery showing giant cells near the fragmented internal elastic membrane (arrow), along with medial and adventitial inflamma- tion. (B) Elastic tissue staining demonstrating focal destruction of the internal elastic membrane (arrow) and associated medial attenuation and scarring.  CHAPTER 7 Diseases of Blood Vessels 113 Takayasu Arteritis bu oers become quescen ater 1 o 2 years and are compabe w Takayasu arteritis is a granulomatous vasculitis of large-sized ong-erm sur vva, abe w vsua or neuroogc decs. arteries characterized by ocular disturbances and weakened Polyarteritis Nodosa pulses in the upper extremities. Takayasu arers maness w ransmura scarrng and cken- Polyarteritis nodosa is a systemic vasculitis of medium-sized mus- ng o e aora—parcuary e aorc arc and grea vesses—and cular arteries that commonly involves renal and visceral vessels umna narrowng o e major branc vesses. Is eaures overap w and usually spares the lung. ose o gan ce arers e dsncon beween e wo enes s made argey on e bass o a paen’s age, w ose younger an 50 Pathogeness. he cause o poyarers nodosa s unknown, bu years o age beng desgnaed as avng Takayasu arers Lke gan e dsease responds we o mmunosuppressve agens and ere- ce arers, Takayasu arers appears o be caused by a cronc gran- ore s beeved o ave an mmunoogc bass. One rd o paens uomaous T-ce–medaed mmune response o an unknown vesse ave cronc epas B necon and may ave crcuang mmune angen. compexes composed o vra angens and specc anbodes, wc depos n vesse was and provoke an nlammaor y response. Wy mmune compexes ocaze n medum-szed areres and no n oer Morphology. he aora, e aorc arc, and s brances, as we as vesses s unknown. e pumonar y, rena, and coronar y areres, may be afeced. he akeofs o e grea vesses may be narrowed or oberaed (Fg. Morphology. Vesses o e kdney, ear, ver, and gasronesna 7.11), eadng o upper exremy weakness and an carod puses. rac vesses are afeced n descendng order o requency. Inlammaor y nraes n vesse was range rom nonspecc Fuy deveoped esons are composed o segmena ransmura accumuaons o T ces and macropages o granuomaous necrozng nlammaon o medum-szed or, occasonay, sma nlammaon, bo o wc may be assocaed w wa ckenng areres, oten w supermposed romboss. Acue esons ave and bross. brnod necross o e vesse wa and nrang neurops (Fg. 7.12), wereas oder esons are broc and assocaed w cronc Clncal Features. Invovemen o e aorc arc and major brances nlammaon. Acue and cronc esons coexs, suggesng produces reduced upper-exremy bood pressure and puse sreng, ongong recurren nlammaor y damage. he vascuar njur y may neuroogc decs, vsua ed deecs, and bndness. Sympoms mpar e peruson o downsream ssues, eadng o uceraons, reaed o nvovemen o e dsa aora (eg caudcaon), e pu- narcs, and scemc aropy, or may provoke aneur ysm ormaon monar y arer y (pumonar y yperenson), e ear (myocarda and e aendan rsk o emorrage. narcon), and e rena areres (sysemc yperenson) aso may appear. he dsease as a varabe course. Some cases rapdy progress, Clncal Features. Poyarers nodosa s more common n young adus bu can occur a any age. he cnca course ypcay s epsodc, w ong sympom-ree ner vas. Ina sympoms—maase, ever, and weg oss—are reaed o nlammaon and are nonspecc, and e vascuar nvovemen s vared and may be wdey dsrbued, eadng o puzzng, proean manesaons suc as yperenson due o rena arer y nvovemen and abdomna pan and boody soos caused by gasronesna esons; dfuse muscuar aces and pans; and perp- era neurs, predomnany afecng moor ner ves. Rena nvovemen s a major cause o dea. Unreaed, poyarers nodosa s usuay aa, A B Fig. 7.11 Takayasu arteritis. (A) Aortic arch angiogram showing reduced flow of contrast material into the great vessels and narrowing of the brachiocephalic, carotid, and subclavian arteries (arrows). (B) Cross sec- Fig. 7.12 Polyarteritis nodosa, associated with segmental fibrinoid tions of the right carotid artery from the patient shown in (A) demon- necrosis and thrombotic occlusion of a small artery. Note that part of the strating marked intimal thickening and luminal narrowing. The white vessel (upper-right, arrow) is uninvolved. (Courtesy Sidney Murphree, circles correspond to the original vessel wall; the inner core of tan tissue MD, Department of Pathology, University of Texas Southwestern Med- is the area of intimal hyperplasia. ical School, Dallas.) 114 CHAPTER 7 Diseases of Blood Vessels bu w mmunosuppressve erapy remsson or cure s aceved n res—probaby due o nvovemen o nerves. I e paen connues o 90% o cases. smoke, cronc uceraons may deveop and progress over me o gan- grene. Smokng absnence can a progresson, bu once esabsed, Kawasaki Disease e vascuar esons do no rem w smokng absnence. Kawasaki disease is an acute, febrile, self-limited illness of infancy Small-Vessel Vasculitides and childhood associated with an arteritis of large- to medium- sized vessels. Many oer orms o vascus prmary afec sma vesses. Aoug Is cnca sgncance sems rom requen nvovemen o coro- er paogeness s ncompeey undersood, wo groups ave emerged: nar y areres.    Antneutrop cytopasmc antbody (ANCA)–assocated vascu- tdes. hese vascudes are dened by e presence o auoan- Pathogeness. he cause o Kawasak dsease s unknown. he dsease bodes agans varous componens o neurop granues. here sows a seasona varaon n ncdence, suggesng a a vra nec- are wo orms o ANCA: (1) an-proenase-3 ANCA (ormery on rggers an mmune response a njures areres secondary. reerred o as c-ANCA), dreced agans PR-3, a neurop azuro- pc granue consuen (PR3-ANCA); and (2) an–myeoperox- Morphology. he vascus resembes a seen n poyarers dase ANCA (ormery reerred o as p-ANCA), dreced agans nodosa, aoug brnod necross s usuay no as promnen. a neurop ysosoma enzyme, myeoperoxdase (MPO-ANCA). Afeced vesses ave a dense ransmura nlammaor y nrae a Severa orms o ANCA-assocaed vascudes are recognzed w ncudes neurops and mononucear ces. I et unreaed, damage paray overappng eaures. hey ncude PR3-ANCA posve caused by e nlammaon may ead o aneur ysm ormaon. granuomaoss w poyangs and MPO-ANCA posve mcro- scopc poyangs. Bo are assocaed w gomeruoneprs and Clncal Features. Kawasak dsease ypcay maness w conjuncva pumonar y dsease, requeny eadng o emopyss. I s uncear  and ora eryema accompaned by bserng, eryema and edema o e e ANCAs ave a drec paogenc roe, bu ese anbodes are ands and ee, a desquamave ras, and cervca ymp node enarge- presen n a g racon o afeced paens and ereore are o men. Approxmaey 20% o unreaed paens ave cardovascuar com- dagnosc vaue. Treamen s w mmunosuppressve agens. pcaons, mos noaby e deveopmen o coronary arery aneurysms,    Immune-compex, sma-vesse vascutdes. hese vascudes are wc may rupure or rombose, eadng o myocarda narcon and/ caracerzed by mmunogobun and compemen deposon or sudden dea. he dsease responds we o annlammaory agens a e ses o vascuar njur y. Anbodes may bnd drecy o (speccay, nravenous mmunogobun and asprn), wc sarpy componens o e vesse wa or be deposed as par o mmune reduce e ncdence o sympomac coronary arery dsease. compexes, or exampe n assocaon w sysemc upus er ye- maosus. he dsrbuon o vascuar njur y and e resuan cn- Thromboangiitis Obliterans (Buerger Disease) ca ndngs dfer dependng on e naure o e auoanbodes or Thromboangiitis obliterans is a severe form of vasculitis that is mmune compexes. In sysemc upus er yemaosus and Good- strongly associated with tobacco smoking. pasure syndrome, e kdney s severey afeced (see Caper 11). I prmary afecs sma and mdde-szed areres o e arms and Infectious Vasculitis egs, and oten produces severe scema, uceraons, and gangrene and ampuaon o afeced ssues. Drec vascuar nvason by necous agens, usuay bacera or ung, parcuary Pseudomonas, Aspergus, and Mucor spp, may cause Pathogeness. Bo e naon and progresson o romboangs ocazed arers. Vascuar nvason can be par o a ssue necon oberans depend on exposure o obacco smoke, bu e precse roe o (e.g., bacera pneumona or adjacen o abscesses), or, ess commony, smokng s uncear. One dea s a some componen o obacco smoke may arse rom emaogenous spread o bacera durng sepcema drecy damages e endoeum and a e vascuar canges are due or embozaon rom necve endocards. Vascuar necons can o drec oxcy. Aernavey, a reacve compound n obacco smoke weaken arera was suiceny o cause aneur ysms (mycotc aneu- may mody vesse wa componens and nduce an mmune response. rysm) or can nduce romboss and narcon. Ceran HLA-ypes are more suscepbe, conssen w a roe or mmune njury. Ceran enc groups (Israe, Indan subconnen, Jap- DISORDERS OF VEINS anese) are aso a greaer rsk, ponng o one or more genec acors. Varcose vens and rombopebs accoun or a eas 90% o cn- cay reevan venous dseases. Morphology. Caracerscay, ere s segmena acue and cronc ransmura vascus o medum- and sma-szed areres, Varicose Veins predomnany n e exremes. In e eary sages, mxed Varicose veins are abnormally dilated tortuous veins produced by nlammaory nraes, wc may gve rse o mcroabscesses, are chronically increased intraluminal pressures and weakened vessel accompaned by umna romboss. he nlammaon oten exends wall support. no conguous vens and nerves (a eaure rarey seen n oer orms Venous daon can occur a mupe ses: o vascus). W me, romb organze and evenuay e arery    Varcose vens o e exremes occur n up o 20% o men and one and adjacen srucures become encased n brous ssue. rd o women; obesy and pregnancy ncrease e rsk. hey mos commony afec e superca vens o e egs. Varcose daon renders e venous vaves ncompeen and eads o ower-exremy Clncal Features. Paens are usuay younger an 45 and ave a sass, congeson, edema, pan, and romboss. Perssen edema smokng sory o more an 20 pack-years. hromboangs ober- and secondar y scemc skn canges may ead o sass dermas ans may presen w Raynaud penomenon, nsep oo pan nduced and uceraons, wc oten ea poory and are prone o superm- by exercse, or superca noduar pebs (venous nlammaon). he posed necon. vascuar nsuicency ends o be accompaned by severe pan—even a  CHAPTER 7 Diseases of Blood Vessels 115    Esopagea varces may arse n e seng o pora yperen-    Capary emangomas are e mos common ype; ese occur n son secondar y o ver crross and (ess requeny) pora ven e skn, subcuaneous ssues, and mucous membranes o e ora obsrucon or epac ven romboss (see Caper 13). Esop- caves and ps, as we as n e ver, speen, and kdneys (Fg. agea varces are one o severa ypes o porosysemc suns a 7.13A). Hsoogcay, ey are composed o n-waed capares may appear w pora yperenson; oer common ses o sun- w scan sroma (Fg. 7.13B). ng ncude e reca vens (emorrods, descrbed beow) and e    Juvene emangomas (so-caed strawberry emangomas) o e perumbca vens (producng wa s known as caput medusa, a newborn are common (1 n 200 brs), nvove e skn, and may ancu erm or e magned resembance o e daed snake- be mupe. hey grow rapdy, bu mos regress compeey. ke vens o e ead o Medusa). Esopagea varces are cncay    P yogenc granuomas manes as rapdy growng, red peduncu- sgncan because ey are prone o rupure, eadng o massve, aed esons on e skn or gngva or ora mucosa a beed easy somemes aa upper gasronesna emorrages. and are oten uceraed. Mcroscopcay, ey resembe exuberan    Hemorrods are varcose daons o e venous pexus a e ano- granuaon ssue (Fg. 7.13C). reca juncon. hey resu rom proonged pevc vascuar conges-    C avernous emangomas are composed o arge, daed vascuar on, mos commony due o pregnancy or sranng o deecae. cannes (Fg. 7.13D). C ompared w capar y emangomas, Hemorrods are a source o beedng and are prone o romboss cavernous emangomas are more nrave, requeny nvove and panu uceraon. deep srucures, and do no sponaneousy regress. hey may be ocay desrucve; surgca excson may be requred n some Thrombophlebitis cases. Cavernous emangomas are one componen o von Hp- Thrombosis of deep leg veins accounts for more than 90% of cases pe-Lndau dsease (Caper 17), n wc vascuar esons are of thrombophlebitis and is the source of most pulmonary emboli. commony ound n e cerebeum, bran sem, rena, pancreas, Oer ses were deep venous romb may orm are e perpros- and ver. ac venous pexus n maes and e pevc venous pexus n emaes, as Kaposi Sarcoma we as e arge vens n e sku and e dura snuses (especay n e seng o necon or nlammaon). Peronea necons and cer- Kaposi sarcoma is a vascular neoplasm caused by human her- an condons assocaed w ypercoaguaby (e.g., poycyema pesvirus-8 (HHV-8, also known as Kaposi sarcoma herpesvirus vera; see Caper 9) may ead o pora ven romboss. [KSHV]). Kapos sarcoma (KS) s mos common n ndvduas w mpared Pathogeness. hromboss s reaed o one or more componens o e T-ce uncon. Four orms o KS, based on popuaon demograpcs Vrcow rad: low abnormaes, endoea njury or dysuncon, and rsks, are recognzed: and ypercoaguaby (see Caper 3). Proonged mmobzaon a    C  a ss c KS o cc urs n o d e r men o Me d  e r r an e a n , M d d  e resus n venous sass (e.g., exended bed res n e possurgca sae, E aser n, or E aser n Eu rop e an d e s c e n ( e sp e c  a  y As  ke n a z  or ong pane or auomobe rps) ncreases e rsk o deep venous Je w s ). A  e re d  m mu n  y s susp e c e d n a e c  e d  n d  v  du a  s , romboss (DVT) o e eg vens, as do acors causng ypercoagu- bu ov e r  T- c e    m mu n o d e   c  e n c y s n o pre s e n. I m a n  e s  s aby (e.g., magnancy and nered deecs, suc as acor V Leden; as re d - pu r p  e sk n p  a qu e s or n o du  e s , usu a  y on  e  ow e r see Caper 3). Mae sex and age oder an 50 years aso are assocaed e x  re m   e s. W   me  e  e s  on s m ay  n c re a s e n sze and w ncreased rsk. nu mb e r, bu  e y usu a  y re m a n c on   n e d o  e sk n an d sub - c u an e ou s  ssue. Clncal Features. DVTs produce ew reabe sgns or sympoms. Loca    Endemc Afrcan KS ypcay occurs n HIV-negave cdren and manesaons ncude edema, redness, sweng, and pan. In some young adus and can oow an ndoen or aggressve course. I cases, pan can be eced by pressure over afeced vens, squeezng oten nvoves ymp nodes. A severe orm, w promnen vscera e ca musces, or orced dorslexon o e oo (Homan sgn). How- nvovemen, occurs n cdren and s vruay aways aa. ever, sympoms oten are absen, especay n bedrdden paens, and    Transpantaton-assocated KS occurs n sod-organ ranspan e absence o ndngs does no excude DVT. In many cases, e rs recpens n e seng o T-ce mmunosuppresson. I pursues an manesaon o DVT s a pumonar y embosm (see Caper 3). To aggressve course and oten nvoves e ymp nodes, mucosa, and preven recurren DVT, ancoaguan reamen s gven, wc may vscera. Lesons oten regress w aenuaon o mmunosuppres- be emporar y or eong, dependng on e eoog y o e DVT. son, bu a e rsk o organ rejecon.    AIDS-assocated (epdemc) KS s an AIDS-denng ness and remans e mos common HIV-reaed magnancy wordwde. TUMORS OF BLOOD VESSELS AND LYMPHATICS he ncdence as aen more an 80% n popuaons w access Tumors o bood vesses and ympacs ncude bengn emango- o anrerovra erapy, bu KS s occurs n HIV-neced nd- mas (exremey common), ocay aggressve neopasms a measa- vduas w an ncdence a s 1000-od ger an n e gen- sze nrequeny, and rare, gy magnan angosarcomas. Vascuar era popuaon. AIDS-assocaed KS oten nvoves ymp nodes neopasms may arse  rom e endoeum or ces a suppor or and dssemnaes wdey o vscera eary n s course. surround bood vesses. Prmar y umors o arge vesses (aora, pu- monar y arer y, and vena cava) occur nrequeny and are mosy Pathogenes s. KS s an unusua neopasm a key begns as a reac- sarcomas. ve proeraon smuaed by acors produced by HHV-8 neced sroma ces. hese ncude vra omoogs o c yoknes suc as ner- Hemangiomas eukn 6, as we as vra acors a enance ce c yce progresson Hemangiomas are common benign tumors composed of blood- and nerere w e uncon o umor suppressors suc as RB. In lled vessels. e absence o an efecve T-ce response, s proeraon per- Severa varees o emangomas are recognzed, ncudng ypes a sss; w me and cona evouon,  may progress o a u-ledged occur preerenay n newborns: magnanc y. 116 CHAPTER 7 Diseases of Blood Vessels Pathogeness. Angosarcomas can arse n e seng o ympedema Morphology. Cu aneous esons progress  roug   ree s e quen a (e.g., oowng ymp node resecon or breas cancer), radaon, and, s ages: patc, paque, and nodue. Paces are pnk, red, or pur pe rarey, ndweng o oregn bodes (e.g., srapne). Hsorcay, poyv- mac ues comp os ed o daed, r regu ar, and anguae d bo o d ny corde as been assocaed w angosarcoma o e ver. vess es ass o caed w  an n rae o cronc nlammaor y ces. Paques (Fg. 7.14A) are ee vaed esons comp os e d o daed der ma vas c uar cannes ned and sur rounded by Morphology. In e skn, angosarcomas rs appear as sma, pump spnde ces. No duar esons are over  y neopas c and sarpy demarcaed, red nodues. Advanced esons are arge, lesy con an ncre as ed numb ers o pump, proera ng spnd e ces, red-an o gray-we masses (Fg. 7.15A) w -dened margns oten w  nersp ers ed s- ke spaces (Fg. 7.14B). he no duar a bend mpercepby w surroundng srucures. he exen o s age oten s accompaned by no da  and vs cera nvovemen, dferenaon s varabe, rangng rom umors a orm vascuar par c uary n  e A r can and AIDS-ass o cae d var ans. cannes (Fg. 7.15B) o undferenaed spnde ce umors wou dscernbe bood vesses. Clncal Features. he course o KS vares wdey accordng o e cn- ca seng. Cassc KS s argey resrced o e surace o e body, and surgca resecon usuay s adequae reamen. In KS assocaed Clncal Features. Oder adus are more commony afeced, and esons w mmunosuppresson, resoraon o T-ce uncon by reducng mos oten nvove e skn, sot ssue, breas, and ver. Angosarcomas mmunosuppresson oten s efecve. Smary, or AIDS-assocaed are aggressve umors a nvade ocay and measasze. Compee KS, anrerovra erapy generay s beneca. resecon oten s no possbe, and curren 5-year sur vva raes are ony abou 30%. Angiosarcomas Angosarcoma s a magnan neopasm dened by evdence o endo- ea dferenaon. A B C D Fig. 7.13 Hemangiomas. (A) Hemangioma of the tongue. (B) Histologic appearance in juvenile capillary hemangioma. (C) Pyogenic granuloma of the lip. (D) Histologic appearance in cavernous hemangioma. (A and D, Courtesy John Sexton, MD, Beth Israel Hospital, Boston. B, Courtesy Christopher D.M. Fletcher, MD, Brigham and Women’s Hospital, Boston. C, Courtesy Thomas Rogers, MD, University of Texas Southwestern Medical School, Dallas.)  CHAPTER 7 Diseases of Blood Vessels 117 A B Fig. 7.14 Kaposi sarcoma. (A) Characteristic coalescent cutaneous red-purple macules and plaques. (B) Histo- logic view of the nodular stage, demonstrating sheets of plump, proliferating spindle cells and slit-like vascular spaces. (Courtesy Christopher D.M. Fletcher, MD, Brigham and Women’s Hospital, Boston.) A B Fig. 7.15 Angiosarcoma. (A) Angiosarcoma of the right ventricle. (B) Moderately differentiated angiosarcoma with dense clumps of atypical cells lining distinct vascular lumina.