HUMAN PATHOLOGY
Volume 22. No. 6 (June 1991)
sies on XT patients with L’2 ( 18%) showittg massive sickling and cerebral infhrcts. 1.3 (13%) having massive sickling of cerebral vessels withour proven infitrcts. and the remaining 83 (69%) containing multiple vessels with sickled cells but no impactions. Thus , 30% of. S(ZpI‘patients had signiticant sickling and may have had central net-votes system symp totns. Patients with SCJ‘ have had slow, progressive infarction of‘ the ftraiti and sf)irtaf cod,"? f>ituitnr!;. and retina.“’ Hemoglobin AS is ttot an innocuous ~enotvpe. Karl\ et al’ 1 have recentfv shown 2111 increased itt&len& of’ sucfdett death in SCT re&tits. High altitude encatnpn~et~t, exercise, and flying without comf)ressiott are acceflted causes of sickling in heterozygous disease. Many military personnel are exposed to all three of‘ cite above hazards. The visual hallucinations without paranoid ideation were a cfue to sickling. The cerebraf comf~ottettt of’ sickle cell anemia and Xl‘ cntphasi03 bilateral watershed areas with sluggish circulation (relative hyf”)f’et.fitsiott), stagttaCon, and the potential for sickling. Subcortical white tnatter in the occipital pole is most frequently involved,” and is the most logical site for \,isual hallucinations of’ organic origin. Frontal pole, centrum ovalr, internal capsule, and potts follow in descending order of‘ fl-ecfuency as sites of’ isc-ltemia and infarction. The laboratory ref’ort of “hrtiiol~sis” of’ tfte hfood was reasonable in view of‘ elevated serum l~o~assiutii level and pigmentation. However. it wax not due to f’ftfehr)tottt);, ~rallSpOr~ }mddeiilS. 01‘ hr:lliIlg of’ the bhd. .h’ sickle CTsis led to apparettt disseminated intravasculat. coa,gulatiott. and infarction of multiple organs (liver, spleen, etc.) c-ausecl fysing of their cells. Repeat blood santpfes showed progressive elevation of‘ serum potassiunt tt) 7.5 mg!dL., the E:I( used, their specificittes, and sources are listed itt ‘l’ahl~ I Control sections were irlc-uhated with buff’er or rtonteactive mouse mortoclonal at~tihody in place 01 primal-\ antibody. Positive control sect ions of‘ lvmph nc)de, tonsillat. tissue, or atien(,c~trc-inotrta izrre ittcuhated with each arttiboclv. Stained tissue’ sections were rvalua~ed I)v two pathoiogists (A.(;.M. .mci R.D.(;.) for staining intensity and cell tapes rractittg with each antiboci\.
I‘he ptletll is .I 4%vear-old white mart who cirveloped sharp, severe, ittlerniittrnt right back attd flank pin wliic-lt hr initiallv attributed to 4 rtx ut.rertt kidney stone. In the f’ollowittS:\\rek?,‘,~~~~s lhe ptirnt sustained a ‘10-11)weight loss. atrot-&a, and fatigue. An at~rtc~rr~ial intravenous p~t4o~qrarrt Irci IO a ~.ctntp~ter-toed alxit~t~~irtal tomogtqhic cs;tnt. whic,h t-cvealeci a pam r-ratic- tti;is~. .I’hv pat ien\‘s p;t>t medic-al history was posirivv IOI- glauc( ma dtid c2tat’dct 01 the lrft eye. tight tre~~~irc~litliiasis, ltiat4 her-nia, mci :t I c~ettt t-ight inguinal het,niot.~ltal’lt~. He tkttirci jaunciic-r, naustx, and vomiting. Ott pltysic;tl e~atiiiIiation ltis sclerae were iionicteric~ ,tiId the right attci leit sul~t‘~~~ia\,ic~itla~fossae were tree 01 a~lenopatli~. ‘l‘hr abclc~trten was sott, with nor-niai active bowrl souncis. ditd 2 IO-c-m mass was readilk palpateci in the cpigastriurtt. No lte~~;ttos~~ienc~rnegai~ or periuniMi~;ti I\ tll~,lladrtlo~,;tr ll\ \vas appt-ecktecl. A ret tal ex,ttiiinatiott iIItretrIarhat~ie. On ,tdtitixsioti tlie patient had a ncnmal complete l~ic~od c ouit1 Cutci coagulaliori l~aratrtetcr-s. Other iabot-atot-Y values ittchIcit4 (~~Iorm;~ls tn parentheses): blooci urea nitrogen 20 tttgitil. (7-20). c rratirtint. 1.1 tng/cil, (0.5 I A), lotal t~ilitut~irt 0.6 rn~$dI. (0.S 1.X), lac-tate dehylro~enast 1-K 1U (,X19-f), scruni ~littatriic~-osalc,;icetic transantirtab~ 177 IU (C3.5). alk,tlittr pltosphatasc 36-l 11‘ (3%I 3 1). total protritt ti.i g/cilA (~!i..S-i.!)). aticf alf)urriiti 4.3 g/dL (Y.O-4..5). I\ chest s-rav tx3e;1lrci no ;icti\e c~tt~ciic~~~itlrrtor~~tr~~ ciisrase and tie rvicfettcr of riirtast~iti~ tlisea\c. ‘l‘liv ptirrtt uttdrt-weiir ‘* posterior celiac ganglion block, whit h W,IYhtghlv successtui in r-educing the intensit! r,t’ the bath pin. Oti e~pioratc~rv laparot~mtv, a Iaye mass iii tltr Iowt~t- pot-lion of t hr head trf‘ the panc.reas xiti rtncitiaie prx1(3zss \%;ib foitttcl. ‘I‘hr hods and tail of the pancrtxs ;tppea~-ed ittttirr;tteci I,\ c hrottic p~ncreatitis. ‘I‘hrrc was rto tGdencr of in\ asioti of the inferior vena cav:~. superior tnesrnterk vessrl\. pot~1;1l b ein. or li,qatiicnt of i‘reitz; no1 were Ikei- ntt~txilast3 or c~t~~~itton~;rtosis present. ‘Ike p;itient rtnci~:~twrtrt .t patic real i~otittotlerie~lotrt~ with an endto-end ~~;ttt~t~~~tti~~~jejrtttc~slc~t~i~,an end-to-side hepatoje~lltloslotlt\. dnti ait end-to-side ~astrc~jrjrtrtosrotri~ w’ith I1U11( al ~
Volume 22. No. 6 (June 1991)
sies on XT patients with L’2 ( 18%) showittg massive sickling and cerebral infhrcts. 1.3 (13%) having massive sickling of cerebral vessels withour proven infitrcts. and the remaining 83 (69%) containing multiple vessels with sickled cells but no impactions. Thus , 30% of. S(ZpI‘patients had signiticant sickling and may have had central net-votes system symp totns. Patients with SCJ‘ have had slow, progressive infarction of‘ the ftraiti and sf)irtaf cod,"? f>ituitnr!;. and retina.“’ Hemoglobin AS is ttot an innocuous ~enotvpe. Karl\ et al’ 1 have recentfv shown 2111 increased itt&len& of’ sucfdett death in SCT re&tits. High altitude encatnpn~et~t, exercise, and flying without comf)ressiott are acceflted causes of sickling in heterozygous disease. Many military personnel are exposed to all three of‘ cite above hazards. The visual hallucinations without paranoid ideation were a cfue to sickling. The cerebraf comf~ottettt of’ sickle cell anemia and Xl‘ cntphasi03 bilateral watershed areas with sluggish circulation (relative hyf”)f’et.fitsiott), stagttaCon, and the potential for sickling. Subcortical white tnatter in the occipital pole is most frequently involved,” and is the most logical site for \,isual hallucinations of’ organic origin. Frontal pole, centrum ovalr, internal capsule, and potts follow in descending order of‘ fl-ecfuency as sites of’ isc-ltemia and infarction. The laboratory ref’ort of “hrtiiol~sis” of’ tfte hfood was reasonable in view of‘ elevated serum l~o~assiutii level and pigmentation. However. it wax not due to f’ftfehr)tottt);, ~rallSpOr~ }mddeiilS. 01‘ hr:lliIlg of’ the bhd. .h’ sickle CTsis led to apparettt disseminated intravasculat. coa,gulatiott. and infarction of multiple organs (liver, spleen, etc.) c-ausecl fysing of their cells. Repeat blood santpfes showed progressive elevation of‘ serum potassiunt tt) 7.5 mg!dL., the E:I( used, their specificittes, and sources are listed itt ‘l’ahl~ I Control sections were irlc-uhated with buff’er or rtonteactive mouse mortoclonal at~tihody in place 01 primal-\ antibody. Positive control sect ions of‘ lvmph nc)de, tonsillat. tissue, or atien(,c~trc-inotrta izrre ittcuhated with each arttiboclv. Stained tissue’ sections were rvalua~ed I)v two pathoiogists (A.(;.M. .mci R.D.(;.) for staining intensity and cell tapes rractittg with each antiboci\.
I‘he ptletll is .I 4%vear-old white mart who cirveloped sharp, severe, ittlerniittrnt right back attd flank pin wliic-lt hr initiallv attributed to 4 rtx ut.rertt kidney stone. In the f’ollowittS:\\rek?,‘,~~~~s lhe ptirnt sustained a ‘10-11)weight loss. atrot-&a, and fatigue. An at~rtc~rr~ial intravenous p~t4o~qrarrt Irci IO a ~.ctntp~ter-toed alxit~t~~irtal tomogtqhic cs;tnt. whic,h t-cvealeci a pam r-ratic- tti;is~. .I’hv pat ien\‘s p;t>t medic-al history was posirivv IOI- glauc( ma dtid c2tat’dct 01 the lrft eye. tight tre~~~irc~litliiasis, ltiat4 her-nia, mci :t I c~ettt t-ight inguinal het,niot.~ltal’lt~. He tkttirci jaunciic-r, naustx, and vomiting. Ott pltysic;tl e~atiiiIiation ltis sclerae were iionicteric~ ,tiId the right attci leit sul~t‘~~~ia\,ic~itla~fossae were tree 01 a~lenopatli~. ‘l‘hr abclc~trten was sott, with nor-niai active bowrl souncis. ditd 2 IO-c-m mass was readilk palpateci in the cpigastriurtt. No lte~~;ttos~~ienc~rnegai~ or periuniMi~;ti I\ tll~,lladrtlo~,;tr ll\ \vas appt-ecktecl. A ret tal ex,ttiiinatiott iIItretrIarhat~ie. On ,tdtitixsioti tlie patient had a ncnmal complete l~ic~od c ouit1 Cutci coagulaliori l~aratrtetcr-s. Other iabot-atot-Y values ittchIcit4 (~~Iorm;~ls tn parentheses): blooci urea nitrogen 20 tttgitil. (7-20). c rratirtint. 1.1 tng/cil, (0.5 I A), lotal t~ilitut~irt 0.6 rn~$dI. (0.S 1.X), lac-tate dehylro~enast 1-K 1U (,X19-f), scruni ~littatriic~-osalc,;icetic transantirtab~ 177 IU (C3.5). alk,tlittr pltosphatasc 36-l 11‘ (3%I 3 1). total protritt ti.i g/cilA (~!i..S-i.!)). aticf alf)urriiti 4.3 g/dL (Y.O-4..5). I\ chest s-rav tx3e;1lrci no ;icti\e c~tt~ciic~~~itlrrtor~~tr~~ ciisrase and tie rvicfettcr of riirtast~iti~ tlisea\c. ‘l‘liv ptirrtt uttdrt-weiir ‘* posterior celiac ganglion block, whit h W,IYhtghlv successtui in r-educing the intensit! r,t’ the bath pin. Oti e~pioratc~rv laparot~mtv, a Iaye mass iii tltr Iowt~t- pot-lion of t hr head trf‘ the panc.reas xiti rtncitiaie prx1(3zss \%;ib foitttcl. ‘I‘hr hods and tail of the pancrtxs ;tppea~-ed ittttirr;tteci I,\ c hrottic p~ncreatitis. ‘I‘hrrc was rto tGdencr of in\ asioti of the inferior vena cav:~. superior tnesrnterk vessrl\. pot~1;1l b ein. or li,qatiicnt of i‘reitz; no1 were Ikei- ntt~txilast3 or c~t~~~itton~;rtosis present. ‘Ike p;itient rtnci~:~twrtrt .t patic real i~otittotlerie~lotrt~ with an endto-end ~~;ttt~t~~~tti~~~jejrtttc~slc~t~i~,an end-to-side hepatoje~lltloslotlt\. dnti ait end-to-side ~astrc~jrjrtrtosrotri~ w’ith I1U11( al ~
