- Email: [email protected]
“Reversible” functional asplenia in combined immunodeficiency
125
Case reporrs 35% in the first 4 hr; this allows detection viously unsuspected renal abnormalities.
of pre-
V~AV M. DHAWAN D. BEATTYCRAWFORD RICHARD P. SPENCER Departments of Nuclear Medicine and Radiology, University of Connecticut Health Center, Farmington, CT 06032, U.S.A.
References PARR C. H., CLASSMAN L. M.. THOMPSONN. L. et al. J. nucl. Med. 14, 534 (1973). VIERAS F. and BOYD C. M. J. nucl. Med. 16. 1109 (1975). SPIGOS D. G., TAN W., PAVEL D. G. et al. Am. J. Roentg.
129, 409 (1977).
DHAWAN V. M., SZIKLAS J. J., SPENCER R. P. and GORDON I. J. Clin. nucl. Med. (in press).
International Journal of Nuclear Medicine and Biology Vol. 5. pp. 125- 127 0 Pergamon Press Ltd 197X Printed in Great Brltam
“Reversible” Functional Asplenia Combined Immunodeficiency*
Case
Report
A female infant was first seen at the Dempsey Hospital at age 3 months. She was admitted for evaluation of thrombocytosis and the presence of HoweIlL Jolly bodies in the peripheral blood. The child was born after a normal full term pregnancy. The mother (27). father (34), and a 6-yr old sister were all in good health. The patient had been previously diagnosed as having combined immunodeficiency disease with associated adenosine deaminase deficiency. Blood values revealed IgA under 4 mg/dl (N: 71-264) IgG 386 (N: 922-1704), and IgM 19 (N: 28-306). Bone marrow biopsy revealed hypercellular marrow with an M : E ratio of 5 : I; there were plentiful megakaryocytes and occasional siderocytes. Cytogenetic study showed 46 X,X chromosomes, consistent with a normal female karyotype. A liver scan (99mTc-sulfur colloid intravenously) revealed the hepatic length to be approximately 6.5 cm. A functional spleen could not be identified. The child was seen again at age G months following several admissions for pneumonia (including one episode associated with Pneumocystis carinii). This second visit was necessitated by vomiting and loose stools of 2 days duration. The liver tip was slightly palpable. Stool cultures, and examination for ova and parasites, were both negative. At this time the red cell morphology was normal, and no Howell-Jolly bodies were present. A repeat liver scan (Fig. 1) revealed the hepatic shape to be unchanged. Just below the left upper quadrant extension of the liver. a small area of additional functional tissue was seen. Multiple angulated views showed this to be distinct from the liver. The patient was referred for thymic transplantation.
in Discussion
(Receiced
15 November
1977)
THE OCCURRENCE of functional asplenia (anatomic presence of the spleen without the ability to accumulate intravenously administered radioactive colloid) has been noted in several diseases!‘.” The usual situation is the onset of the splenic defect in childhood or adult life, and the irreversibility of the functional asplenia once it occurs. There is gathering information that some cases of functional asplenia may have the disorder at least temporarily reversible. The first evidence was that in some very young children with sickle cell disease, functional asplenia could be transiently reversed by transfusions with nonsickling red cells.‘3~4’The second set of data was that in certain newborns with cyanotic congenital heart disease, a hyposplenic state was present at birth but tended to revert toward normal later in life.“’ Finally, Baum and coworkers have presented evidence that individuals with hemoglobin S-C disease may have episodes of splenic hypofunction perhaps related to transient vascular blockade.@’ We wish to report another disorder associated with reversible functional aspleniacombined immunodeficiency. * Supported by USPHS National Cancer Institute.
Grant
CA 17802 from the
At age 3 months this child (with combined immunodeficiency) had no demonstrable speen on the radiocolloid scan, and Howell-Jolly bodies were present in the peripheral blood. At age 6f months, HowellJolly bodies were no longer demonstrable, and radiocolloid images showed the presence of a small functional spleen. The initial bout of functional asplenia might have been triggered by vascular blockade related to the thrombocytosis, or to the malabsorption. Why this should reverse at age 6; months is uncertain. However, combined immunodeficiency has to be added to the small list of disorders in which functional asplenia can apparently be reversed. It is of course uncertain as to whether this is a cyclic phenomenon (as in hemoglobin S-C disease). RICHARD P. SPENCER KOLLI SURESH HOWARD
A.
PEARSON
SAVITA
Department of Nuclear Medicine, University of Connecticut Health Center, Farmington, CT 06032 and Department of Pediatrics, Yule-New Haven Medical Center, New Haven, CT 06.504
PLJRI
126
Case reports
References 1. PEARSONH. A.. SPENCERR. P. and CORNELIUS E. A. New Engl. J. Med. 28, 92>926 (1969). 2. SPENCER R. P., PEARSONH. A. and BINDER H. A. J. nucl. Med. 11, 763-766 (1970). E. A., 3. PEARSON H. A., SPENCERR. P., CORNELIUS TREVESS. and LANGER. C. J. nucl. Med. 11, 349-350 (1970). 4. PEARSON H. A., CORNELIUSE. A., SCHWARTZ A. D., ZEL~ON.I. R., WOLFSONS. L. and SPENCER
5. 6.
R. P. New Engl. J. Med. 283, 334-337 (1970). PEARSONH. A., SCHIEBLER G. L. and SPENCER R. P. Pediatrics 48, 277-280 (1971). J~~PHEG., ROTHENBERG S. P. and BAUMS. Am. J. Med. 55. 72&722 (1973).
of the sternal notch, slight epigastric tenderness, and a palpable liver edge. Hemoglobin was 15.1 g/dl, WBC 20,000 with 88% polys, 6% bands, 6% lymphocytes. Liver function tests were normal. A chest X-ray showed an anterior mediastinal mass (with extension toward the sternal notch). A liver-spleen scan was performed with 99T~m sulfur colloid; it revealed a rounded defect principally involving the right upper hepatic lobe (Fig. 1, left). Scintiphotos were obtained 48 hr after i.v. administration of 67Ga citrate. There was accumulation in the upper abdomen and mediastinum (Fig. 1, center). Indeed, the major accumulation corresponded to the hepatic defect noted on the radiocolloid scan. Uptake of radiogallium in the lesion was so intense that there was relatively little in the liver itself. Superimposition of the 67Ga image on the hepatic defect on the radiocolloid scan could be seen (Fig. 1, right).
0047-0740/78/0601-0126SO2.00/0 International Journal of Nuclear Medxine and Biology Vol. 5. pp. 126-128 0 Pergamon Press Ltd 1978. Printed in Great Britam
Radiogallium Accumulation in a T-Cell Lymphoma* (Received 18 October 1977) BY WAY of contrast to the known affinity of poiymorphonuclear leukocytes for radiogallium, uptake can also occur in lymphocyte derived tumors. We have had the opportunity to study an unusual disorder, a convoluted T-cell lymphoma, which showed intense accumulation of radioactivity after intravenous administration of 67Ga citrate.
Discussion It is now recognized that lymphomas may be derived from various components of the immune system.‘” Although cases of T-cell lymphoma have been described,“) such instances are unusual. We are not aware of a prior report of radiogallium studies in a T-cell lymphoma. The present case suggests that such tumors have an avidity for radiogallium. This is of interest since the rate of positive radiogallium scans is usually lower in the lymphomas than in Hodgkin’s disease.@’ The case also raises the interesting point of whether the abnormal lymphocytes accumulate the radiogallium, or if the radiolabel is concentrated by support elements or other associated cells. Apparently the massive uptake in the abdominal and mediastinal tumor acted as a “sink” for radiogallium, with little available for concentration in the normal liver. VIIAY M. DHAWAN MOWED A. ANTAR RICHARD P. SPENCER? SAVITA PURI
Case Report A 22-yr old male had previously presented with a right sided pleural effusion and a mediastinal mass. Thoracentesis yielded a fluid with a white cell count of 3,500 per mm3 with 97% lymphocytes. Both a cell block and a pleura1 biopsy revealed the morphology of a nearly monocellular convoluted T-cell lymphoma. The cells were further characterized by immunological and ulatrastructural characteristics. The huffy coat of a centrifuged specimen of peripheral blood yielded identical cells. The patient was begun on vincristine, adriamycin and prednisone, followed by mediastinal radiation. The present admission was brought about by an intermittent dull epigastric pain of several days duration. Physical examination revealed an afebrile white male in no acute distress. Pertinent findings included a small mass to the left * Supported by USPHS Grant CA 17802 from the National Cancer Institute. t To whom requests for reprints should be addressed.
University of Connecticut Health Center, Farmington, CT 06032, U.S.A. Veterans Administration Hospital, Newington, CT, U.S.A.
References BERARDC. W., GALLOR. C., JAFFEE E. S., GREEN I. and DEVITA V. T. Jr. Ann. intern. Med. 85,
351 (1976). GREENBERGB. R., PETERC.
R., GLASSYF. and
MACKENZIEM. R. Cancer 38, 1602 (1976). GREENLAWR. H., WEINSTEIN M. B., BRILLA. B., MCBAIN J. K., MURPHYL. and KNISELEYR. M. J. nucl. Med. 15. 404 (1974).
Case reporrs 35% in the first 4 hr; this allows detection viously unsuspected renal abnormalities.
of pre-
V~AV M. DHAWAN D. BEATTYCRAWFORD RICHARD P. SPENCER Departments of Nuclear Medicine and Radiology, University of Connecticut Health Center, Farmington, CT 06032, U.S.A.
References PARR C. H., CLASSMAN L. M.. THOMPSONN. L. et al. J. nucl. Med. 14, 534 (1973). VIERAS F. and BOYD C. M. J. nucl. Med. 16. 1109 (1975). SPIGOS D. G., TAN W., PAVEL D. G. et al. Am. J. Roentg.
129, 409 (1977).
DHAWAN V. M., SZIKLAS J. J., SPENCER R. P. and GORDON I. J. Clin. nucl. Med. (in press).
International Journal of Nuclear Medicine and Biology Vol. 5. pp. 125- 127 0 Pergamon Press Ltd 197X Printed in Great Brltam
“Reversible” Functional Asplenia Combined Immunodeficiency*
Case
Report
A female infant was first seen at the Dempsey Hospital at age 3 months. She was admitted for evaluation of thrombocytosis and the presence of HoweIlL Jolly bodies in the peripheral blood. The child was born after a normal full term pregnancy. The mother (27). father (34), and a 6-yr old sister were all in good health. The patient had been previously diagnosed as having combined immunodeficiency disease with associated adenosine deaminase deficiency. Blood values revealed IgA under 4 mg/dl (N: 71-264) IgG 386 (N: 922-1704), and IgM 19 (N: 28-306). Bone marrow biopsy revealed hypercellular marrow with an M : E ratio of 5 : I; there were plentiful megakaryocytes and occasional siderocytes. Cytogenetic study showed 46 X,X chromosomes, consistent with a normal female karyotype. A liver scan (99mTc-sulfur colloid intravenously) revealed the hepatic length to be approximately 6.5 cm. A functional spleen could not be identified. The child was seen again at age G months following several admissions for pneumonia (including one episode associated with Pneumocystis carinii). This second visit was necessitated by vomiting and loose stools of 2 days duration. The liver tip was slightly palpable. Stool cultures, and examination for ova and parasites, were both negative. At this time the red cell morphology was normal, and no Howell-Jolly bodies were present. A repeat liver scan (Fig. 1) revealed the hepatic shape to be unchanged. Just below the left upper quadrant extension of the liver. a small area of additional functional tissue was seen. Multiple angulated views showed this to be distinct from the liver. The patient was referred for thymic transplantation.
in Discussion
(Receiced
15 November
1977)
THE OCCURRENCE of functional asplenia (anatomic presence of the spleen without the ability to accumulate intravenously administered radioactive colloid) has been noted in several diseases!‘.” The usual situation is the onset of the splenic defect in childhood or adult life, and the irreversibility of the functional asplenia once it occurs. There is gathering information that some cases of functional asplenia may have the disorder at least temporarily reversible. The first evidence was that in some very young children with sickle cell disease, functional asplenia could be transiently reversed by transfusions with nonsickling red cells.‘3~4’The second set of data was that in certain newborns with cyanotic congenital heart disease, a hyposplenic state was present at birth but tended to revert toward normal later in life.“’ Finally, Baum and coworkers have presented evidence that individuals with hemoglobin S-C disease may have episodes of splenic hypofunction perhaps related to transient vascular blockade.@’ We wish to report another disorder associated with reversible functional aspleniacombined immunodeficiency. * Supported by USPHS National Cancer Institute.
Grant
CA 17802 from the
At age 3 months this child (with combined immunodeficiency) had no demonstrable speen on the radiocolloid scan, and Howell-Jolly bodies were present in the peripheral blood. At age 6f months, HowellJolly bodies were no longer demonstrable, and radiocolloid images showed the presence of a small functional spleen. The initial bout of functional asplenia might have been triggered by vascular blockade related to the thrombocytosis, or to the malabsorption. Why this should reverse at age 6; months is uncertain. However, combined immunodeficiency has to be added to the small list of disorders in which functional asplenia can apparently be reversed. It is of course uncertain as to whether this is a cyclic phenomenon (as in hemoglobin S-C disease). RICHARD P. SPENCER KOLLI SURESH HOWARD
A.
PEARSON
SAVITA
Department of Nuclear Medicine, University of Connecticut Health Center, Farmington, CT 06032 and Department of Pediatrics, Yule-New Haven Medical Center, New Haven, CT 06.504
PLJRI
126
Case reports
References 1. PEARSONH. A.. SPENCERR. P. and CORNELIUS E. A. New Engl. J. Med. 28, 92>926 (1969). 2. SPENCER R. P., PEARSONH. A. and BINDER H. A. J. nucl. Med. 11, 763-766 (1970). E. A., 3. PEARSON H. A., SPENCERR. P., CORNELIUS TREVESS. and LANGER. C. J. nucl. Med. 11, 349-350 (1970). 4. PEARSON H. A., CORNELIUSE. A., SCHWARTZ A. D., ZEL~ON.I. R., WOLFSONS. L. and SPENCER
5. 6.
R. P. New Engl. J. Med. 283, 334-337 (1970). PEARSONH. A., SCHIEBLER G. L. and SPENCER R. P. Pediatrics 48, 277-280 (1971). J~~PHEG., ROTHENBERG S. P. and BAUMS. Am. J. Med. 55. 72&722 (1973).
of the sternal notch, slight epigastric tenderness, and a palpable liver edge. Hemoglobin was 15.1 g/dl, WBC 20,000 with 88% polys, 6% bands, 6% lymphocytes. Liver function tests were normal. A chest X-ray showed an anterior mediastinal mass (with extension toward the sternal notch). A liver-spleen scan was performed with 99T~m sulfur colloid; it revealed a rounded defect principally involving the right upper hepatic lobe (Fig. 1, left). Scintiphotos were obtained 48 hr after i.v. administration of 67Ga citrate. There was accumulation in the upper abdomen and mediastinum (Fig. 1, center). Indeed, the major accumulation corresponded to the hepatic defect noted on the radiocolloid scan. Uptake of radiogallium in the lesion was so intense that there was relatively little in the liver itself. Superimposition of the 67Ga image on the hepatic defect on the radiocolloid scan could be seen (Fig. 1, right).
0047-0740/78/0601-0126SO2.00/0 International Journal of Nuclear Medxine and Biology Vol. 5. pp. 126-128 0 Pergamon Press Ltd 1978. Printed in Great Britam
Radiogallium Accumulation in a T-Cell Lymphoma* (Received 18 October 1977) BY WAY of contrast to the known affinity of poiymorphonuclear leukocytes for radiogallium, uptake can also occur in lymphocyte derived tumors. We have had the opportunity to study an unusual disorder, a convoluted T-cell lymphoma, which showed intense accumulation of radioactivity after intravenous administration of 67Ga citrate.
Discussion It is now recognized that lymphomas may be derived from various components of the immune system.‘” Although cases of T-cell lymphoma have been described,“) such instances are unusual. We are not aware of a prior report of radiogallium studies in a T-cell lymphoma. The present case suggests that such tumors have an avidity for radiogallium. This is of interest since the rate of positive radiogallium scans is usually lower in the lymphomas than in Hodgkin’s disease.@’ The case also raises the interesting point of whether the abnormal lymphocytes accumulate the radiogallium, or if the radiolabel is concentrated by support elements or other associated cells. Apparently the massive uptake in the abdominal and mediastinal tumor acted as a “sink” for radiogallium, with little available for concentration in the normal liver. VIIAY M. DHAWAN MOWED A. ANTAR RICHARD P. SPENCER? SAVITA PURI
Case Report A 22-yr old male had previously presented with a right sided pleural effusion and a mediastinal mass. Thoracentesis yielded a fluid with a white cell count of 3,500 per mm3 with 97% lymphocytes. Both a cell block and a pleura1 biopsy revealed the morphology of a nearly monocellular convoluted T-cell lymphoma. The cells were further characterized by immunological and ulatrastructural characteristics. The huffy coat of a centrifuged specimen of peripheral blood yielded identical cells. The patient was begun on vincristine, adriamycin and prednisone, followed by mediastinal radiation. The present admission was brought about by an intermittent dull epigastric pain of several days duration. Physical examination revealed an afebrile white male in no acute distress. Pertinent findings included a small mass to the left * Supported by USPHS Grant CA 17802 from the National Cancer Institute. t To whom requests for reprints should be addressed.
University of Connecticut Health Center, Farmington, CT 06032, U.S.A. Veterans Administration Hospital, Newington, CT, U.S.A.
References BERARDC. W., GALLOR. C., JAFFEE E. S., GREEN I. and DEVITA V. T. Jr. Ann. intern. Med. 85,
351 (1976). GREENBERGB. R., PETERC.
R., GLASSYF. and
MACKENZIEM. R. Cancer 38, 1602 (1976). GREENLAWR. H., WEINSTEIN M. B., BRILLA. B., MCBAIN J. K., MURPHYL. and KNISELEYR. M. J. nucl. Med. 15. 404 (1974).