Senile amyloid of heart, 2, Carcinoid syndrome, hecrt. 3. Hurler`s

LOUISIANA PliTHOLOGY SOCIE'fY
GULF RSGION, COLLEGE OF AJ.1ERICAN PJ.THOLOGISTS
DIAGNOSES
1,
Senile amyloi d of heart,
2,
Carcinoid syndrome , hecrt.
3.
Hurler ' s disear;e, he<:.rt
4.
Non specific aortitis.
5.
Aortiti s ,
6,
Scleroderma, lung ,
7.
8,
Scleroderma , kidney.
9,
Periarteritis nodose , secondar y, lung ,
10 ,
Gicnt cell angi itis , kicmey,
11 ,
Lupus er ytl1emato sus, kidney,
12 ,
Subocute glomerulonephrit i s ,
13.
Acceler <ted n rtGrioloncphr osclerosis .
14 .
t"ioschco1-ri.t z 1 s dise.:.se , kidney,
15 ,
Focr:l
rhewnat~id
arthritis,
Periarteritis no dosa, kidney.
11
embolic 11 glomerulonephritis,
February 1961
•
LOUISIANA PATHOLOGY SOCIETY
and
GULF COAST REGION 1 COLLEGE OF AMERICAN PATHOLOGISTS
SE~ITNAR
ON CONNECTIVE TISSUE AND COLLAGEN TISSUE DISEASES
February 17, 18 and 19 1
1.261
CASE 1. A 78 year old Negr ess had mild diabetes for 16 years. For
one year she noted shortness of breath and·ankle edema . Six months
ago there was sudden ri ght- sided paralysis, and thereafter, she
remained bed-tidden, Drowsiness and genernl deterioration caused
her e.dmission.
There we::"El marked dehydration, clouded senso riUII!l1 right facial weakness .:md hemiplegia . Temperat ure, 1000; pulse, 100; r espirations,
24 ; BP 90/60. The lungs contained sca ttered basilar rales , The ·
heart was moderat ely enlarged ,but there were no murmurs , A large 1
multinodular uterus· filled· the lov1er abdomen. ·
·
Laboratory: RBC, '3 . 4; hb, 1 9 . 5 gm; hematocrit, 30; \lBC 1 14,500 ~
Urine , albumin 4t 1 reducing substance 3f, aceto ne positive . BUN,
100 mgm; FBS, 216 mgm; · C02 18 mEq, Electrocardiogram shov1ed nonspecific abnormalities .
\'lhile being transported to the X-ray department the patient suddenly
became apneic, pulseless and was declared dead 10 minutes later ,
CASE · 2 .
A 69 year old house1otife ent ered because of lower abdominal
Three years a go she was opere.ted upon for intestinal obstruction, and an inoperable bowel tumor was treated by a small intestine by-pass .· A nodule in the liver was removed for biopsy and
sho1~ed c F.rcinoid,
FollOI•ring operatinn she noted a graducl onset of
intermittent diar rhea with abdominal cramps, spells of nervousness,
and a periodic flu3hing of the f a ce and hands , For one y~ar there
was progressive dyspnea a nd pal pitation of increasing severity.
Examination showed o. well developed but cachectic woman ~lith a curious
reddish , blue , blotchy appearance of the 'f ace . Temperature, 990 ;
pulse, 110; r espirations, 28; BP 120/70. The lungs were clear, the
heart l'tas slight l y enl a.rged, rapid, and forceful. There was a reduced
pulmonic second sound and a soft diastolic murmur at the right' border
of the sternum. The liver extended down 5 fingerbre adths, was
slightly tender and was questionably nodular, The abdomen was moderately distended ,
·
·
·
·
Laboratory: Hb 1 16 gms; Hct 50%; IJBC, 11 1 500, 65% neutro !?hils , A
urine examinatlon for 5 hydroxytryptamine 1·1as positive,
Shortly after entry , during a bout of severe dys pnec:t , the r:a tient
di ed .
pa1n,
1
l CASE 3. A 4 year old ~Thite girl was born of 49 year old parents; a
Ji3 year old sibling was said to ha ve been operated upon f or a cranial
J synostosis . At birth the r:atient seemed normal, but at one year
of age there was r etardation of movement and speech . At the t ime
of entry she was still bottle- fed and kllew very fe1~ h'O r ds . For 10
days the pati ent had a cold; on the day before entry there were high
fever, anorexia,and a rash on the face and trunk , Shortness of
...:2-
CASE 3; Continued
breath a nd cyanosis of th e lips o;md fingertips
ensued.
Physical examination s howed an a cutely ill, lethargic girl Nith·
gr o.t e sque facial features and a morbilliform r ash , Ter.lpera ture,
102°; pulse, 120; respire.tions, 38. The head -v1as large and ro t•nded ;
there were prominence of the orbital ridges and a depressed nasal
bridge, The lips 1·1ere heavy and thick, The teeth were separated
and the gingiva thickened, Chest barrel-shaped, There was a pot
belly with a small 1 em umbilical hernia. The liver extended do 1-m
4 fingcrbreadths and the spleen 3 £ingerbreadths,' There was ankylosis
of the glenohumeral joints. The hands were broad,and the f ingers
small and contracted,
·
·
I Laboratory: WBC 10 1 000; 71% polys, Urine negative.
I Because of dyspnea, oxygen was administer ed .
1
Shortly after entry ,
it was noted that the heart bea t trias rapid and weak , Despite
digitali za tion the patient died a day later,
CASE 4.
A 22 year old college girl complained of dyspnea fo r one
year. 'Tv1o ye a rs ago ~Then enterin g college, she \•TRS told t hat she had
a "bad heart 11 and was p l aced on digitalis , Thi s she continued to ·
take until t "VIO we.e ks ago when it was discontinued because of nause a .
1
As a ch ild she had frequent sore throats, but neither arthrit is· nor
chorea , There \•Tas no edema , but the liver and spl een were enla rged
for 1 year , Four days before entry· cough and mild left chest pain
1were followed by nausea, r e tching, and loose stools, Just before
entry dyspnea became se.v ere, and the J:atient a ppeRred letha rgic.
Tempera ture, 99,6°; pulse, lZO and regul a r; res pira t i o ns, 36; BP
1
170/20, She was acutely ill but not orthopneic. Petechia e were noted
1
'· in the lower conjunctivae, Neck veins were r.wderately distended,
~ and there wa s ma:r)<ed carotid pul sation ,
The lungs were clear.
The hee.rt wa s enlarged to the anterior <tx i llary line in the sixth
inte rspac e . Forcef ul pre·cordial pulsations were manifest. A lo11d
to and fro murmur was best heard in the third l eft interspace and at
/ tlie xyphoid; it was transmitted all over the pr ecordium. The.re was
no thrill , The liver 1•1a s enlarged and slightly tender. The spleen
was
· not felt, Nailbeds ~1ere clear; not cyanotic,
'·
1
RBC , 4.4 million; hb, 10; '1'/ BC 15,700 with 76% polys. Urine, albumin
4(., · no sugar; sediment containe d many \IIBC ' s. Serum bilirubin 0.35/0/9
mgm. Several serclogic tests for syphilis during the preceding t1•1o
years and at this entry 11ere negative , Electroca rdiogram showed
L, A,J;l, Vli'.;h non-specific alterations , Rhythm was r e gular.
Digi tali zahon Wc.s rei11 sti tuted, but on the second ·hos pital day ankl e
edema \'las noted . Death occurred suddenly and unexpectedly on th e
following day,
I
CJ\SE 5. A 57 year old white man suffered from polya rthritic rheumatoid arthritis for 6 yea rs . This re sponded inte rmittently eJld
'variously to steroid therapy . He h ad been unab l e to v10rk for five
.years because of crippling deformities. Among ot h er features he had
had several attacks of episc l eritis, A year before entry he devel oped
pleur al ei'fu sion a nd a di ffuse lower lobe pul monary infiltra te ~;ith
relatively few symptqms, An open chest bi.opsy s !10\·1ed a gr anulomatous
pleurit i s considered consistent ~lith rheumatoi d disease . Th ree months
~efore·' entry 2n aortic systolic murmur ~1as heard, and 3 months later
this was accompanied by manifesta t ions of an aortic insufficiency,
~ymptoms of hea rt f ailure s uperven ed a nd continued relentlessly to
death.
-3-
CASE 6~ A 67 year o l d vlhite man 1·12s dead on arrival , He had had
§l coug for 7 ye ars, and 3 years ago he consulted a ?hysician for
cough and exertiont.l c~ynpne a , Thickening , contraction , a nd pigmentt>.ti on of the skin on the face, torr:o, an d a rms led to a · diagnosis
of scleroderma. At th&t time the bl ood pressure was 160/$0 1 and
chest film was s nicl to be norma l. Cortisone caused considerabJ.e
improvement of s:rmptoLls, Nine mm<ths ago cough nnd shortness of
breath recurred , This time ro emt genogra.ms revealed di ·f fuse pulmonary
infiltration pe.rticull:\rly · i n the right upper l obe, Antibiotic·therapy
caused r elief of symptoms , <'l.nd he was dischar ged after 3 1<1eeks, although we<>.k and dyspneic. T1·1o months before his death he l•ras dig-·
itali zed becau.se of hear t fni l ure, At that time the ·thickening and
pi gmentati on of the skin had·increo;sed and now involved the lower
extremities , Blood pressure, 140/85, Che.st film exhibited i nc r eased
peribronchia l markings 1-rith infiltrate th r ou ghout both lower lung
fields. A b arium s l'lallow s hm•red r e t <trdation of f'lo1·1 through the
esopha gus , He was discharged 8fter i mproveme nt. On the d<ly of death·
while driving his auto in city traffic, he- suddenly died at the l•meel.
CASE 7. A 57 year old white male was fir st a dmitted to · the hospi tc>.l
four months before death 1·r ith a compluint of tight skin, dating back
six months . The ill ness began v1ith edema of the hands a nd a di agnosis
of scleroder ma was made, He 11as treated with ach elating· agents" vthich
resul ted in "softening of the skintr . · At the fir st· entry~ blood '
pressure '1-l~s 180/90; BUN, 60 mgm; FBS, 127 mgm; hb., lO,o gm; i'lBC,
15,900 1'1ith 84% neutrophile. Serologic tests for syphilis 1·1ere
nega tive. He wns·dischr rge d but r eturned t o the hospital 4 days
before fer dyspnea . Blood pr essure 1~as now 210/120, Pulse, 110;
respi ra ~;io n s, 34.
The patient was orthopneic and exhi bited stridor .
The h eart. was enlarged, Fundi s :.1owed soft exudates and. flume- shaped
hemorrhages •Nith to:rtuous vessels. There was l'o'lttr}(ed thickening of
t h e skin qn the extremiti es and fc.ce, · ·
·
·
Lab? r<-~ory: · RBC, 2. 5; hb,, . 7 !3 gm; WBC, 8, 900 1 s o tlium 1 146 mEq;
potass ~ um, 5.4 mEq. A/G = 3.2/) . 5 gm.
Shortly nfter e ntry a shock-like state ( BP 120/60) 1'ras stabilized
v1i th oxygen and levophed . Later, however; renal shutd01•m occurred ,
The BUN rose to 112 mgm and the co2 fell to 23 mEq, Creatinine rose
to 12.4 mgm, Cal cium, 5 mEq; phosphorus, 3 . 6 mEq; potassi um, 6.3 mEq.
Afte r 2 da ys be lapsed i nto coma and di ed .
l
1
CASE 8. A 69 year old wh i te female was admitted .as a d i sposit ion
'))roblem , The history was poor. For 3 1veeks there hD.d been polyuria,
polydip:Jia, dry thro a t, a norexia and \•re ight l oss of 15 lbt For - two
hours before entry she had com:?lained of· d i ffuse abdomi n<:t l pain.
'
Physical examinntion:·tcmpe r r ture, 99.2°, BP, 165/80; pulse, irregula r,
120; r espir .:.t-ions , 22.. The patien t was dehydrated a nd emac:!ated ,
The hea rt was modera tely enle>.rged, nnd there was fi brilJ.::t.:fun . 'l.'he
abdomen 1iras essenti al l y nor mal. There was edemE>. of the l o1·rer extr ei tie ::: . Deep ten don· r e flexes wer e r educed.
Labor atory:- \IBC , · 23 , 400; polys 68%, lymp,hs 22 , monos 10 ; h b., 9gm.
U):"ine, SPG 1.015, albumin 1/, a cetone 21, no s ugar . The sediment
cont::_i ned moderate numbers of red and Nhite cells. Electrocardi ogram
exhi bited fi .b rillat i on 1·1ith f i rst degree otrioventric ular block, FBS,
210 mgm. . BUN rose from 40 to 90 mgm. A/G : 1.7/2 .. 9 gm.
Supportive therapy '~>las given but served no p urPose , She continued
to complain Of e bdominal pain a n d was found deac1 in bed on the tenth
hos:Ji ta.l day.
-4CASE 9. A Z5 year o l d \•lhite nur se com~Jl<l ined of dyspnea on exer t i on.
She
been kho1m to h ave he&rt murmur s sinc e age 1 5 yee.ri:J , Her
mot her had had <1 virus infecti on .durin g the third trimester of
pregnancy. There was no h i stor y of sce,rlet or rheumatic fever • Vfuel!D
examined 10 yectrs earlier , BP was 110/60, A split, firs t sound 1 a
Grade II systolic murrnl.lr c>.nd m e!;>r ),y blo wi ng di astolic were heard
i n the 3r\i and 4th left i nterspe,c es. ::;KG showed severe R. A. D. and
r ight ventricular hypertrophy. Six yet>rs bef~re entry the p~tient
\'las digi talized because of e xerti ona l dyspnea an d 5 yea rs ago sh e \•11:\S
gi ven mercurial diuretics , At about t hi s time a cute ba ct<Oori nl endocarditis (Staphylococcus aur e us) ,.,a s t r eated successfully with
penicillin . During the next 4 years she wo rked a s a nur s e but had a
recur rence of the s epticemia vdth fever 1~ years ago; again this wa s
corrected with penicill in.
C<?~rdiac c a theteri zation revealed inc r eased pulmonary a rt 0r i a l p r essur e ,
X- r a ys exhibited pulmonn ry ov ercir culGti on wi th a h ilar d<mce, i\n
·
angiogram r ev ealed a prominent left atrium ~nd evidence of a small
int erventricul<~r septal defect .
Shortly a f t':lr the a:.~:iogram ther e wer e se·izures , on.c~ she succumbed i n
a sh ort intervul.
naa
CASE 10. A 76 year old Negro 1·10me,n had enl urgi ng masses in the left
ingui nal region fo r 6 months, Shortly before entry t hes e becnme
ulcer a ted end bled, For two months there had been progressi ve wee.kne <l S confi ning her to bed. ·
Physica l exl?'.min;?.t ion : :)ul se, 120; B P 90/60; respirations , 30 ;
temperature, 101 o. The pati ent vms cachectic <~1 thou gh i n no rout~
distress . Several irregul~r ul cera tions in the l e ft ingui nal re gion
measured up to 6 em. Th e l iver was EJoderate1y enlarged. A sm&.ll mass
•.vas palpable just i nsi de the anal verge.
A bio psy of both the anal mass and t he groin ulc era.tj. ons showed undifferentiated squamous c<'-rcinoma . X- ray t h er2 py was admi nistered ,
t.tnd th e i ngui nal le sian became consi derably smaller. She we.s di s phar ged improved · but retuTned · vdth severe diarrhe<'. . At thi s time the
j1e mogl obin was 5.5 gm ; \!fBC, 9 ,100. The uri ne con t a ined l '"rge numbers
of red and vihit e blood cells. The dia rrhea \•iaS quite seve1'C 2nd
~s soyiated with rectc>l bleeding ,
A shock- like s t R;;e s uperve ned and
t;he died 24 hours after ent r y.
CASE 11. A 24 yeer old Negress hnd a n i n termittent f ac i o.l r c sh f or
0Yee.rs, This was partic u l arly bad when exposed to s unl i ght , For
4 years there "'er e r ecurrent epi sodes of a rthralgi a · especi a lly of the
$ma ll joint :; of the h<:.nds , She wa s found to have L. E . cells in a bone
l]la rro w p r eparatj.on. · Serolog i c t est for syph i l is was p ositi.v e , BUN
23 mgm; A/G : 3.0/J,O. Recently there •,;ras severe abdomina l pa i n and
for 24 hours marked n a use a and vomiting~
·
PhysicA.l ext>J•lil'lations: l'emperL~ ture, 102,2°; p ulse , 108 ; respire> tions ,
24; BPt 140/90. The r e 1-1as a butterfly ro.sh on the face, The abuomen·
wa_s distended, and there vrere diminished bowel s ounds . · Se1'um amylase J
52; BUN, 20 mgm; co 19 mEq ; soclium, 125 mEq; ;Jotass ium, 4 . 4 mEq , ' Li v er
floccol 2.tio n tests 2\·rer e positive , and total bilirubin was o. 8 mgm.
\'lBC 8; 100. Urine con t ain ed
albumin and many red and l'lh.ite blood
cells.
Al t hough abclomin<:~l pa i n appe?.red to warrant surgery, i t was felt thr'.t
he.r condi t i on was too poor t o . justi fy oper a t i on , Short ly aft,e r entry
the ''IBC rose to 61, 000 11ith 99% neut ro;Jh ils, Lower abdomine.l pain
·
was i ntense, and there v1a s r igi dity. As cites deve loped r <'. ther quickly,
She beca me comatose c.nd Gi ed 8 days a fte r entry.
3t
-5-
CASE 12 . A 2 ye ::.r old 11hite boy was brought to the hospital in vrhe.t
was thought to \:e acute he~ rt f nilure . He hnd hr.d chickenpox one
month before e.nd haC. been exposed to me;.sles two weeks e.zo . Shortly
before entry he wr.s ttken to a phys:i.cianls office "mere a diagnosis of
pneumonia was mde. He"' es given penicillin by needle and 20 minutes
l l'.ter showed evidence of ncute heart failure vlith r apid Heak pulse .
He was brought to the·hospit a l for emergency treat ment .
Epi nephrine , levophed 1 nnd hydrocortisone were admini.stered with on l y
transitory respons e , .;hortness of brenth becrulle intense , Edema of the
lo;Ier extremities appenred 1 t>nd he died 6 hours after entrYo
CASE la· A 27 year old Negress entered the hospi tal complnining of
blurre vision an~ c bdominal soreness . During her first pre~ancy at
age 15 yetrs a diagnosis of hypertension (blood pressure 150(110) was
made , She did not return to the cl inic thereafter and ap,arently got·
alone quite well . ''hen the blurred vision appe;;:red two months befor e ,
t his distu r bed her con sider .!!bly . A week before entry, however, there
wns severe gaseous distention , d i z;ziness and marked t·rea kness.
·
Physical examination~ Blood pressure 230/170 ; pulse,·lOO; ~aspir a tions,
28 ; temperatur e , 980 , The ;:>utient was well develo ped , obese , and ·
lethErgic 11ith an acetone odor to the breath. Fundi showed marroN 1
tortuous a rterioles , both old and recent her:~orrha ge::; r ncl exudr>.t es . The
hea rt was noder?.tely enlerged uith a Grade I I systolic murmur at the
apex. The tbdornen v1~s negr.tive. Dee:;> tendon reflexes were hypoactive .
Laboratory : RBC 2.0, million ; hb. , 7 gm; · UBC 5, 300 "'ith 76% polys .
Urine specific gr<Jvity 1,011 1 illbumi n 2/ 1 acetone if. The sediment
cont<'ined many RBC ' s nnd cast s . BUN 88 rapidly ro s e t o 236· mgm.
C02 1 ll.5 .,mEq; chlo~ide 1 ? 1 mEq; cr~atin ine , 31 mgm; so dium , l20 _mEq ;
·
phosphoru" , 7 . 0 mEq, calc~urn , 4.8 ml!;q.
Evidence of ~meu.11onia ( st;.phyl ococcal) ap:?eared 1 como supervened , and
she die~ one month after entry.
CASE 14. A 35 yer> r old He gress entered complaining of headache . She
hud b een t~ell until three lteeks eL>rJ.i er when fro!!t~~- hea da che occurred
accompanied by nervousness, ill.,.def:ined chest <md 8bdomin<l pain .
There had been menorrhc.gia for one week. For five · days there 1·1as fever
and increasing confusion , na usea and much vomiting . ·
·
Pulse , 112 ; resr)irati ons , 28 ; B? 115/90; tem·)ere>ture 1 104
The
patient was lethargic 11nd rest l ess , There were · e cchymoses on t he c hest
wrll 1 a rm, a nd e. l arge one on the right buttock . · The mucous membr<>nes
\'/ere pale •,Jith scvttcrcd petechiae . Lune;s, heart, end abdomen were
neg<' tive. There v1as a questionable· right facitl ,.1 eclmes:3 .
Laborntory : RBC· l . 7; hb . ,5.5; \TBC 6 , 400 with 53% polys . No abnorm.::l
cells were seen, but th ere 1'/ere no platelets viei ble in the SLJear ,
Clott-ing time . v~ts 7 minutes , bleeding :time over 20 minutes, no clot
retrocti on a t 24 hours . Urine • unremarkable. Hematocrit 14%.
TOurniquet tes t - stron.?;lY positi ve . BUN 14 rngm. S-erum bilirubi n
0 .45/ 3 . 2 mgm .
Transfusion of ;·hole blood was given on the morning after entry ;While
it wr s being ad>ilinistere<:: , respir :ztory C~n cl pulse r ate incrc;. sed , and
the blood pres sure fell to 90/35 . Transfusion was discoa tinued, but
the p;:.t~ent beceme brea.thless, lap!'ed into coma, c.nd died.
°.
CASE 15. A 27 year old 1t!ite \·JOm<m fir Gt entered the hOS.?itnl 9 yeers
'beforeoecause of nn acute exacer bation of rheumati c fever 11ith
evidence of mitra l <.1nd aorti c stenosis., Her fi r st nttack of polyarthritis occur red at aee 12 yenr s. In the hospital t he symptoms
r esponded to s 01licyJ.ates 1 and she r eturned t o wor k. T1·ro yee r s before
- 6c~,~E
.1.2..• Conti.llilec!
her final entry there ~~as swelling of the
alJ~I.ome n and legs .
'i'l,is was improved ~~i t.!J t l::e udi;:ini::.: tration of
dig:.taE s end d'.u~e'Cics. A r ight th rombc)hJ.e!Ji.tis resr,>onded to
<u1 ::.l coa~:;u.lant "...ree;;ment £'. ye<·.r ago .
Fm· three Nee!'s t.h ere v1ere eaema ,
ast:ites , right leb P•'!ir:, shortne :;s 0f ur e :~ th, ro•.l[.il. and hemoptysis, ·
E):mr.:..nation shai•IOd e. wal l ~o-;el::~psd a:::t:_ no:::-: shed, orth:>pna::.e •~man ,
R"l.reral small p:.lstcll es ·:l&!'<l noc.. ed at t he s :. te of a~l <:> bd".'minal par&c envesi a viO und, D~.:l lness and 7·hon~:h i 'iiE:re detected in t he right midl ung f ield . T!·,e hea:-t was ;;Jr,lurged ~o the l.eft wit;h syu~. olic c>.nd
dias -volic ~.lur~.<;.::-s at the base and apex . There· v~ere c>.sc:i.'CP.s and ·
severe pedal t; dema . Temper c:t;;.<re , 99. d0 ; pulse , 62; respirctions,
26; B? 1)0/7().
Labor<.> t ory: Hemoglo bin 10 gn, 'IBC 19 1 300 \'lith $$)~ neutro:,hils ; urine
sho1·1ed 3f oJlbumin, and the sed~.m·::n:t cont<ined <\ le>.r ge number of red
c alls. B:.·~od c:..~ltc.re g:--&•,., n01: he:uoJ yt i.c streptococcus . Bt;i'l, ,fO m01J,
Che s t f ilm rfl•·eale c.! prominence o:· -che left o.trL.IIl! c.nC: puloor.ic con us,
The!'e 1·1<:3 fluid in the :-ight c!:Jeat and a trit-ngule.r density in the
l.'\ . :;.. ::. '4tPl)~r
J.oheo
Hearl; faiJ.ura rem~tined int.r ac r.able, Folj ov1ing ~ni dllin t1·eatment
th e blood ::ul1..ure b!;lc .!tme no gat::. ve . T~ro •·~ales a fte r entry t here i·1as
sudden right chest pain, Rnd the pt'tien ~ died shortly t~ereaft cr ,