Case Reports

FunctionalAsplenia inMalignantMastocytosis

J. Roth, 0. Brudler, and E. Henze

Divisions ofNuclear Medicine and Hematology, University of Ulm, 7900 Ulm, Germany

A 47-yr-oldmanwith maii@antmastocytosis,a m@ant neoplaslaof mastcells, presentedwith anemia.Uftrasonographyrevealedan enlargedspleen.A liver-spleenscan using[9@'―Tc]sulfurcolloldfailedto showanysplenióuptakeconsistentwith the diagnosisof functionalasplenla.Functionalasplensarelatedto this condftlonhasnot beenreported previously.Sincethe spleenwas heavilyinfiltratedwith spaceoccupyingniast cells, a mass c@spIadngeffect alongwith impairedperfusionare discussedas underI@,1ngmechanism.

J NuclMed26:1149—1152,1985

unctional asplenia is defined as inability of the margin and spleen enlargement was estimated by ‘—19X 10 spleen to accumulate technetium-99m (99mTc)sulfur cm. Ultrasonography revealed a distinct enlargement of both colloid administered intravenously, resulting in nonvi liver and spleen (Fig. 1). For the first time circulating mast sualization of the spleen on a liver-spleen scan. First cells were found and a radiograph of the chest showed a left described in patients with sickle cell disease (1), it has sided pleural effusion. Howell-Jollybodies, however,could not be detected in the circulating erythrocytesat any time. been found to be associated with a variety of disorders It wasthought that the anemia might be caused in part by (2—5).In malignant mastocytosis, however, it has not hypersplenism. Therefore, a splenectomy was suggested. Be yet been reported. fore surgery, a liver-spleen scan was performed after intrave nous administration of 2 mCi 99mTc It showed an enlarged CASE REPORT liver with patchy uptake of the sulfur-colloid but no visualizatiQn of the spleen (Fig. 2). A 47-yr-oldwhitemanhadbeenadmittedto the hospital After splenectomy,the patient recoveredand the peripher because of shortness of breath, paleness, and tachycardia. al blood count improved for several months. Histological Laboratorydata disclosedan extremeanemia (Hb 3.9g/lOO examination of the spleen revealed an extensive diffuse infil ml), a thrombocytopenia with a platelet count of 40.000/zl tration of the whole organ with mast cells. Normal splenic and a slightly elevated LDH. All other laboratory results cells were for the most part replaced by monomorphological showed normal values. A computed tomography scan re cells, no reticuloendothelial cells could be visualized. Further vealeda hepatosplenomegaly.A laparoscopyshoweda minor more, there was a complete obstruction of the sinusoidal amount of ascitic fluid and an enlarged but macroscopically spaces resulting in multiple splenic infarctions. The finding unobstrusive spleen. A spleen biopsy showed a normal red and was consistent with the diagnosis of malignant mastocytosis white pulp. Finally, bone marrow aspiration and biopsy led to (Fig. 3). the diagnosis of malignant mastocytosis. The patient wasthen transferred to the universityhospital and presented with severe pain in the left upper quadrant. DISCUSSION UltrasQnography revealed a subcapsular hematoma of the spleen due probably to the previous biopsy. After the symp Functional asplenia, mostly described in sickle cell toms of the hematoma had resolved, an attempt of systemic disease, was rarely also found in other disorders (Table therapy with Eldisine (6) was made without success. The 1). A multitude ofetiological factors has been proposed patient required massive transfusion of erythrocytes during for the lack of radiocolloiduptake in the spleen.Spen all this time. The patient was discharged after reconstitution cer et al. (7) suggestedan initiallyclassificationof the of peripheral blood counts. In the following 6 wk, more than etiology and worked out two main causes of functional ten erythrocytes conserves were given. During the physical asplenia (Table 2): (a) impairment of delivery of the examination the liver was palpable 4 cm below the costal radiocolloid due to circulatory disturbances and (b) Received Feb. 25, 1985; revision accepted July 12, 1985. direct effect on the splenic tissue resulting in FQrreprints contact: Eberhard Hcnze, MD, Div. ofNuclear Medi dysfunction of the reticulo-endothelial system (RES). cine, University of Ulm, 7900 Ulm, FRG. Malignant mastocytosis,a malignantvariant ofgen

Volume 26 •Number 10 •October 1985 1149 FIGURE 1 Ultrasonographyshoweddistinctly enlarged spleen, with size of 18X 10cm

eralized mastocytosis with a primary lack of skin in the distinctly enlarged spleen by mast cells displacing volvement, is characterized by infiltration of visceral the normal cells. organs, in particular bone marrow, spleen, liver and In addition, the extensive infiltration of the enlarged lymph nodes with mast cells (8). Common laboratory spleen with mast cells resulted in a blockage of the findingsare anemia, eosinophiliaand thrombocytope sinusoidal spaces causing multiple splenic infarctions. nia. Splenomegaly observed in most cases is thought to Thus, the impariment ofdelivery ofthe radiocolloid due result in hypersplenism. to reduced blood flow may have contributed to the In the patient described in this report, suppressionof nonvisualization of the spleen. reticuloendothelial cells by mast cells may be a main A final aspect needs to be mentioned, which is the factor for the inability to accumulate [99mTc]sulfur possibility of a selective blockage of the RES responsi colloid. This, in fact, is in accordance with the histologi ble for functional asplenia in part. Hazenberg et al. (9) cal finding which revealed an extensive infiltration of reported a selective blockage of the RES in a case of

anterior view posterior view FIGURE 2 Liver-spleenscanwfthoutvisualizationofspleen.Anteriorviewofliver-spleenscanwithlandmarkspositionedoverrightrib bow.Distanceof landmarkersare5cm,thusshowingliverenlargementof 17cm.Spleenisnotvisualizedonbothanterior andposteriorviews

1150 Roth,Brudler,andHenze TheJournalof NuclearMedicine @ _@$@ @‘ :,@ ,@

@ 1@ ‘@‘@:‘@ •‘@ @ %••‘s@@' ‘a ‘ . -. .4 @ •@r @,

.,q@• 0? @ - .@ • 4 •.- . ‘@. I ‘ ‘I•@‘ d'@ • ‘ @ @:i.st;;,. % 0 ;, • @•@P@'d@“@I @-‘.‘ *% @‘-1'— I @ , I 4. 0@• •4• @. @‘ . @ •@ • $ S

@ . ,. t_,@ •@%@ @ 4,-,: . .% ‘1 @ a.., •@ * ‘or,. @ @‘•b‘ @%4@. #. @ IL@ ‘ .• @, J .u,__ : .@ @. @ . 4 @ I • @•j a

@ 4 . ‘ @- ‘I , FIGURE3 @ ‘@, 9 p @pb ! S'.,. • Spleen specimen of malignant @ # •41'L • @. @. i o - mastocytosls(seetext)

ICondition TABLE Sezarys syndrome which showed splenic uptake of ReferencesSicklewithfunctionalasplenla [99mTcjstannous colloid but not of [@mTcJsulfur col bid. It is speculated that there might be different path (1)Hemoglobincell anemia ways in the accumulation of stannous and sulfur col (2)Cyanoticsicklecelldisease bid. In our patient, no [@mTc]stannous colloid scan (3)Combinedheart dIsease (9)Chronicimmunodeficiency was performed. (5)Sezaryaggressivehepatftis Howell-Jolly bodies, nuclear remnants in circulating (9)Immunoblasticsyn&ome erythrocytes, have been regarded as an indispensable (10)Multiple tymphoma conditionof functional asplenia. In the case reported, (10)Hodgkin'smyeloma (7)FollowingdiseaseafterirradIationor chemotherapy however, Howell-Jolly bodies have never been ob (7)Chronicthorotrast served.Thisphenomenon,describedinlymphoreticular (4)Malignantgraft-versus-hostdisease disorderscombinedwith functional asplenia, is caused mastocytosisTABLE by the extensive bone marrow involvement resulting in myelophthisis (10). The infiltration of bone marrow with mast cells results in a hypoproliferative anemia and thrombocytopeniaand might thus explain the ab sence of Howell-Jolly bodies in our case.

2Etiology ReferencesCirculatoryof functionalasplenia REFERENCES 1. Pearson HA, Spencer RP, Cornelius EA: Functional disturbances asplenia in sickle-cell anemia. N Eng. J Med Splenic(7)Splenicartery blockade 281:923-926, 1969 (7)Sinusoidalveinobstruction 2. Engelstad BL: Functional asplenia in hemoglobin SE obstructiondueblood flow disease.ClinNucI Med 7:100—102,1982. (9)Effectto highviscosity 3. Pearson HA, Schiebler GL, Spencer RP: Functional on splenic tissue hypospleniain cyanoticcongenitalheart disease.Pedi Replacementspleniccellsof normal atrics 48:277—280, 1971 (7)RES by tumors or infiltrates 4. Al-EidMA,Tutschka PJ, Wagner HN, et al: Function (9)Cellularblockadebytoxic agents al asplenia in patients with chronic graft-versus-host fromnutrftionaldamageresufting disease.JNuclMed 24:1123—1126,1983 (10)Selectivedeficiency 5. Dhawan VM, Spencer RP, Sziklas JJ: Reversible func (9)Overstuffingblockadeof RES tional asplenia in chronic aggressive hepatitis. J Nucl of RES (9) Med2O:34-36, 1979

Volume 26 •Number 10 •October 1985 1151 6. Mathe G, et al: Phase II clinical trials with vindesine in Path 67:1—6,1983 patients with hematologicals malignancies. Anticancer 9. HazenbergHJA, Hiddnik HiM, Link EAM, et al: In Res 1:1—9,1981 111 leukoçytescanning and partial functional asplenia 7. Spencer RP, Dhawan V. Suresh K, et al: Causes and in a ptient with Sezary syndrome. Clin Nucl Med temporal sequence of onset of functional asplenia in 8:3—6,1983 adults. Cli,, Nucl Med 3:17—18,1978 10. Gross DJ, Braverman AJ, Koren G, et al: Functional 8. Horny HP, Parawesch MR, Lennert K: Basophilenleu asplenia in immunoblastic lymphoma. Arch Intern Med kaemie und generalisierte Mastozytose. Verh Dtsch Ges 142:2213—2215,1982

1152 Roth,Brudler,andHenze The JQurnalof Nuclear Medicine