Idiopathic thrombocytopenic purpura occurring in a subject previously splenectomized for traumatic splenic rupture: Role of splenosis in the pathogenesis of thrombocytopenia

A 25 year old woman presented with idiopathic thrombocytopenic purpura. She had undergone splenectomy 12 years previously for traumatic splenic rupture. Thrombocytopenia was ultimately resistant to steroid therapy. Howell-Jolly bodies were absent from the peripheral smear and ^99mTC-spleen scan demonstrated foci of increased uptake thought consistent with accessory spleens. However, splenosis alone was demonstrated at laparotomy, and all visible splenotic tissue was surgically removed. The patient responded and adequate platelet counts were maintained after discontinuation of steroid therapy. The functional capacity of splenic implants has been previously demonstrated both in animal and man. However, reports linking splenosis to hematologic disease are rare. In the present case, characteristic splenic function was demonstrated by both the ^99mTc-spleen scan and the absence of the typical peripheral blood findings of asplenia. The hematologic response to the removal of the splenotic tissue attests to its importance in maintaining the thrombocytopenic state. In the setting of prior splenectomy for splenic trauma, splenosis may contribute to hematologic disease. Removal of this splenotic tissue may result in hematologic remission.     

Intrapancreatic accessory spleen. A rare cause of a pancreatic mass.

CONCLUSION: The clinical significance of intrapancreatic accessory spleens resides in the mimicry of pancreatic cancer. Radionuclide tests (Octreotide scan and Tc99m sulfur colloid scan) should be undertaken to distinguish these lesions from neuroendocrine tumors, hypervascular metastases and pancreatic carcinoma. If the tests are equivocal, diagnostic laparotomy or laparoscopy is recommended. BACKGROUND: Despite its relatively common occurrence, intrapancreatic ectopic splenic tissue is rarely detected owing to its asymptomatic nature. METHODS: We report a case of a clinically asymptomatic patient in which abdominal computed tomography (CT) scans revealed a mass of 1.5 cm in diameter in the distal pancreas. The tumor markers CA 19-9 and carcinoembryonic antigen (CEA) were slightly elevated, and pancreatic neoplasm was suspected. RESULTS: Left pancreatic resection and splenectomy were performed. The removed specimen disclosed the presence of an accessory spleen within the pancreatic tail.     

Influence of Residual Splenic Tissue on Autoantibodies in Splenectomized Patients

In 42 patients splenectomized after injury of the spleen or for non-malignant diseases residual splenic tissue was detected by ^99mTc scanning in 19 (Group I) and not detected in 23 (Group II). Autoantibodies detected by the indirect immunofluorescent method occurred in 16 (38%) of the splenectomized patients and in 6 (14%) of 42 age- and sex-matched controls (P < 0.02). In patients with residual splenic tissue the incidence of autoantibodies did not differ significantly from that in the controls, whereas autoantibodies occurred significantly more often in patients without residual splenic tissue (39%) than in the corresponding control group (9%) (P = 0.016). Antibodies to nuclear antigens (ANA), smooth muscle (SMA), reticulin (RetA) and cytoplasm of gastric parietal cells (PA) were detected, and the antibody titres tended to be higher in the patients than in the controls. None of the patients showed any clinical signs of autoimmune disease. These findings indicate that splenectomy disturbs the regulation of autoantibody function, and it is possible that this might be caused by a reduction of the number of T suppressor cells for which the spleen is a major pool. It is suggested that residual splenic tissue may prevent an increased occurrence of autoantibodies in splenectomized patients.     

Heterotopic peripancreatic spleens presenting as possible tumors of the body and tail of the pancreas

Accessory spleens are found in about 10% of routine postmortem autopsies, usually near the hilum of the spleen and the tail of the pancreas. In 16% of cases, the accessory spleen may be found within the pancreatic tail.¹ Its importance arises when computed tomography (CT) scan reveals a tumor in or about the tail of the pancreas. The diagnosis of intrapancreatic accessory spleen may be suggested when the CT scan, following intravenous contrast injection, shows the mass to be enhanced in a manner characteristic of that of a spleen. Accessory spleen can be identified using technetium 99m(^99m)Tc)-labelled heated red cells or^99mTc sulfur colloid.² We report a patient with heterotopic spleens, questioned by CT scan for pancreatic tumors, but accurately diagnosed by^99mTc sulfur colloid scan.     

Intrapancreatic accessory spleen

Summary Conclusion: The clinical significance of intrapancreatic accessory spleens resides in the mimicry of pancreatic cancer. Radionuclide tests (Octreotide scan and Tc99m sulfur colloid scan) should be undertaken to distinguish these lesions from neuroendocrine tumors, hypervascular metastases and pancreatic carcinoma. If the tests are equivocal, diagnostic laparotomy or laparoscopy is recommended. Background: Despite its relatively common occurrence, intrapancreatic ectopic splenic tissue is rarely deted owing to its asymptomatic nature. Methods: We report a case of a clinically asymptomatic patient in which abdominal computed tomography (CT) scans revealed a mass of 1.5 cm in diameter in the distal pancreas. The tumor markers CA 19-9 and carcinomebryonic antigen (CEA) were slightly elevated, and pancreatic neoplasm was suspected. Results: Left pancreatic resection and splenectomy were performed. The removed specimen disclosed the presence of an accessory spleen within the pancreatic tail.     

Preliminary experience of 99mTc-Aprotinin scintigraphy in amyloidosis

Background and aim: Radio‐labelled Aprotinin has been shown to bind with amyloid fibrils in vitro as well as in vivo. The aim was to test the usefulness of ^99mTc‐Aprotinin imaging in systemic amyloidosis. Methods: Thirty‐five cases who had ^99mTc‐Aprotinin scans for the assessment of systemic amyloidosis were reviewed retrospectively. Eighteen had biopsy‐proven amyloidosis and 17 were controls (amyloidosis was excluded by negative biopsies and non‐invasive tests). Five of 18 patients with amyloidosis had final diagnosis of cardiac amyloid. Results: Physiological uptake of ^99mTc‐Aprotinin was noted in the urinary tract (kidneys and bladder) and in the liver of all patients and controls; and non‐specific uptake of ^99mTc‐Aprotinin was visualised in the spleen and oro‐facial structures in the majority of both groups. Myocardial ^99mTc‐Aprotinin uptake was noted in all five patients with final diagnosis of cardiac amyloidosis and in none of the 30 subjects who did not have cardiac amyloid. The median heart to background uptake ratio was 2.0 in cardiac amyloid patients and 1.1 in subjects without cardiac amyloid (P = 0.0004). Single Photon Emission Tomography (SPECT) studies of the thorax confirmed that the site of uptake lay within the myocardium. In the amyloidosis group, site‐specific ^99mTc‐Aprotinin uptake was also identified in the subcutaneous tissue of the legs and in a breast nodule shown to be positive for amyloidosis on biopsy. Conclusions: ^99mTc‐Aprotinin imaging may be a useful non‐invasive method for the assessment of the presence and extent of extra‐abdominal amyloid, particularly cardiac amyloidosis. It has little role in diagnosis of amyloidosis involving the oro‐facial and abdominal structures.     

Evaluation of splenectomy in large granular lymphocyte leukaemia

We performed splenectomy in four patients with severe neutropenia (less than 0.5 X 10(9)/l), recurrent infections, and splenomegaly associated with large granular lymphocyte leukaemia. Prior to splenectomy, elevated levels of neutrophil-reactive IgG were detected in sera of all three patients tested. In all patients, enlargement of the spleen was due to a characteristic lymphoid infiltration of red pulp cords. Splenectomy resulted in an increased neutrophil count greater than 0.5 X 10(9)/l in all patients; this response was sustained in two patients who benefited clinically by a dramatic reduction in frequency of infections. Poor clinical response was associated with elevated levels of antineutrophil antibody post-splenectomy. All four patients had an increase in number of circulating large granular lymphocytes post-splenectomy; one patient who had attained a sustained neutrophil response died of an accelerated lymphoproliferative disorder 19 months post-splenectomy. We conclude that splenectomy may be of value in correcting severe neutropenia and reducing infections in some patients with large granular lymphocyte leukaemia. However, splenectomy appeared to be of no value in treatment of the underlying lymphoproliferative disorder.     

Detection of Splenosis and Ectopic Spleens with 99mTc-Labelled Heat Damaged Autologous Erythrocytes in 90 Splenectomized Patients

Splenosis or ectopic spleens were detected in 22 of 45 patients splenectomized after either abdominal trauma or accidental lesions of the spleen during operation. The incidence of ectopic spleen tissue in various groups of splenectomized patients has been investigated by a sensitive scanning method employing reinjection of ^99mTc-labelled heat damaged autologous erythrocytes. In comparison 7 cases were found among 45 patients who underwent splenectomy for haematological reasons. The time span between the operation and a positive scan varied between 3 months and 11 years. None of the patients in the haematological group with reoccurrence of spleen tissue presented any signs of relapse of their primary disorder. The only patient with overwhelming infection was a girl in whom splemectomy was performed for hereditary spherocytosis. She recovered from the sepsis and her scan was negative. It is concluded that recurrence of spleen tissue is frequent after traumatic lesions of the spleen but rare after selective splenectomy for haematological reasons. This may account for the lesser tendency to overwhelming sepsis after post-traumatic splenectomy.     

Accessory spleen in recurrent chronic immune thrombocytopenic purpura.

From 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat-treated RBC labeled with Tc-99m. Platelet kinetic studies with 51Cr or 111In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearance of bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpura who relapsed or failed after splenectomy.     

The relationship between human spleen and blood erythroid burstforming units (BFU-E)

The influence of splenectomy on erythroid burst colony formation by peripheral blood mononuclear cells from 10 patients (four with hereditary spherocytosis, two with beta-thalassaemia major, two with Hodgkin's disease and two with idiopathic thrombocytopenic purpura) was studied. In every instance splenectomy was followed by a lowering of blood BFU-E. The post-splenectomy levels ranged from 0 to 30% of the preoperative levels. Mononuclear cells from the spleens of eight patients were cultured and found to contain numerous BFU-E. The total quantity of BFU-E in the whole blood and in the spleen of the patients was generally of the same order of magnitude. The number of splenic BFU-E did not correlate with spleen size. Splenic BFU-E differed from peripheral blood BFU-E in that they were more sensitive to erythropoietin (Ep) and in that they failed to respond to burst promoting activity (BPA) produced by preincubating the spleen mononuclear cells with phytohaemagglutinin M (PHA). In contrast, media conditioned by PHA-treated spleen cells contained BPA active on peripheral blood BFU-E from normal individuals. These data suggest that the spleen may have an influence on the numbers and functional properties of BFU-E.     

Quantitative distribution of radiolabeled 5-Aminosalicylic acid enemas in patients with left-sided ulcerative colitis

Rectally administered suspensions of 5-aminosalicylic acid (5-ASA) are topically effective in treating left-sided ulcerative colitis. The extent to which the contents of these enemas are distributed to inflamed mucosal linings has not previously been determined. This study was undertaken to validate a technique for labeling 5-ASA with^99mTc and to quantitate the distribution of [^99mTc]5-ASA in eight patients with left-sided ulcerative colitis. Eight patients underwent three colonic scintigraphic exams within five days, receiving a 60-ml radiolabeled 5-ASA enema into the unprepared rectum for each study, with sequential anterior abdominal images obtained for 4 hr. Activity within the rectum, sigmoid, descending, transverse, and ascending colon was quantitated. Over 50% of the labeled enema had advanced beyond the rectum in five of eight patients and in six of eight patients by 30 min and 60 min, respectively. The distribution of [^99mTc]5-ASA was quantitatively reproducible when repeated in the same patient on different days, despite apparent visual differences. By 2 hr, the amount of the enema present within the rectum decreased significantly (P<0.05) compared to the initial distribution. The amount of enema present within the descending colon was increased significantly at 0.5 hr (P< 0.05) and at 2 hr (P< 0.01). There were no significant changes in the distribution from initial values for the sigmoid, transverse, or ascending colon at any time. In each of these cases the spread of the enema to or beyond the extent of disease was documented. In patients with left-sided ulcerative colitis, small volume [^99mTc]5-ASA enemas reliably reach the area of inflammation.Supported by a grant from Reid-Rowell, Inc.     

Fungal splenic abscess

Nineteen patients with fungal splenic abscesses included 16 with leukemia, one drug user, one patient with Cushing's syndrome, and one without predisposing causes. Fifteen had persistent fever; six had esophageal or mucocutaneous candidiasis. Abdominal pain occurred in seven patients, splenomegaly occurred in six, and left abdominal mass occurred in one. Three chest x-ray films showed left pleural effusions; two had elevated left hemidiaphragms, two had left-sided infiltrates, and one was normal. Sixteen of 17 technetium Tc 99m sulfur colloid scans were abnormal, as were 15 gallium citrate Ga 67 scans. Two computed tomographic studies revealed splenic defects. Candida was the cause in 15 cases, Aspergillus was the cause in three cases, and Blastomyces dermatitidis was the cause in one case. Sixteen patients underwent splenectomy, 12 receiving postoperative antifungal therapy. Fifteen survived regardless of underlying disease or hepatic microabscesses.     

Rates accessoires après splénectomie pour thrombopénie immunologique chez un patient ayant un déficit immunitaire commun variable

Introduction

Blood cells are mainly destroyed in the spleen during autoimmune cytopenia. Amongst the various therapeutic strategies, splenectomy is sometimes necessary during the disease course. However, splenosis or accessory spleens can account for autoimmune cytopenia relapse after initial splenectomy in these patients.

Case report

We report an 18-year-old male with common variable immunodeficiency who presented with immunological thrombocytopenia. Splenectomy allowed remission of cytopenia, but a relapse was attributed to splenosis, because Jolly bodies were absent on blood smear. Laparoscopic splenectomy of accessory spleens induced long term remission. A literature review is performed.

Conclusion

Fifteen to 20% of relapses of autoimmune cytopenia treated by splenectomy are related to accessory spleens. Ablation of accessory spleens can cure again the patients, including patients with accompanying common variable immunodeficiency.     

Accessory spleen: ultrasonographic and tomographic characteristics]

The failure of fusion of splenic tissue results in the formation of accessory spleens. This entity normally appears in post-mortem examinations. The purpose of this work was to determine the incidence of the accessory spleen and to compare the ultrasonographic and tomographic features of the accessory spleen and the main spleen in a group of one thousand patients affected with several abdominal pathologies. Five hundred of these patients were studied by ultrasonography and five hundred patients by computed tomography. The ultrasonographic and tomographic features of the accessory and main spleen were similar. Its diagnosis is specially important in certain groups of patients with hematologic disease or abdominal trauma with splenic tear. This is due to the fact that the hypertrophy of the accessory spleen can produce a relapse of the disease.     

Splenic irradiation for chronic autoimmune thrombocytopenic purpura in patients with contra-indications to splenectomy

Summary We treated by splenic irradiation eight patients with chronic idiopathic thrombocytopenic purpura (ITP, seven cases) or secondary autoimmune thrombocytopenic purpura (one case) who had contra-indications to splenectomy. A total dose of 15 Gy was delivered to the spleen, with left kidney protection. One patient had a good durable response (>1 year); two patients had a good transient response (of 3 months duration) but they responded again to a second course of irradiation; two patients had only partial response, but have required no other treatments for 2 years; the three remaining patients had no response. Side-effects were minor. Therefore splenic irradiation appears to be a therapeutic option in patients with chronic ITP who have contra-indications to splenectomy.     

Clinical utility of (99mTc)‐TRODAT‐1 scans to differentiate Parkinsonian syndromes: case studies and commentary

The differential diagnosis of Parkinsonian syndromes is challenging, especially in the elderly as they often have complex presentations. Nuclear imaging modalities can increase diagnostic accuracy. Single‐photon emission tomography (SPECT) using the ligand (^99mTc)‐TRODAT‐1 is used overseas but is yet to be approved in Australia. Three pilot cases of clinically unclear Parkinsonian syndromes from the Royal North Shore Hospital Aged Care Department who underwent (^99mTc)‐TRODAT‐1 scans are described. From experience overseas, the (^99mTc)‐TRODAT‐1 scan has clinical utility in the differential diagnosis of Parkinsonian syndromes. (^99mTc)‐TRODAT‐1 has a lower cost and greater availability than other ligands. Our initial scan findings are largely consistent with the clinical picture, but low specific binding can affect scan interpretation. (^99mTc)‐TRODAT‐1 is a potential tool in the differential diagnosis of Parkinsonian syndromes. Further experience regarding its effect on patient outcomes and cost effectiveness is required before routine clinical use can be recommended.     

Treatment advances in adult immune thrombocytopenic purpura

Immune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by autoantibody-mediated destruction of platelets. The disease generally runs a mild clinical course, though significant morbidity and mortality can occur. Steroids and/or splenectomy are effective in treating the disease in approximately 70% of patients. These treatments have been well established with approximately 50 years of clinical experience. While open splenectomy is the traditional surgical procedure, laparoscopic splenectomy, splenic artery embolization, and splenic irradiation are viable alternatives. For patients who relapse after the above therapies, treatment is more difficult and seldom results in a cure. The goals of therapy involve maintaining a safe platelet count while minimizing toxicities from the treatment. Multiple treatment options exist including corticosteroids, androgens, immunomodulatory drugs, cytotoxic chemotherapy, immunoglobulin preparations, bone marrow transplantation, Helicobacter pylori eradication, and others. While the standard treatment of steroids and splenectomy has changed little over the past decades, a number of promising new therapies on the horizon may soon join the armamentarium upon which the clinician can draw to fight the disease. In this review, we will examine treatment for chronic ITP in adults in the pre-splenectomy, splenectomy, and post-splenectomy settings.     

Foamy Histiocytosis of the Spleen in Patients with Chronic Thrombocytopenia

In 17 of the 64 spleens removed from patients with chronic thrombocytopenia lipid-laden histiocytotes were demonstrated. No correlation was found between these pathological findings and the age of the patients at diagnosis or splenectomy, the duration before splenectomy of therapy with glucocorticoids, the period with thrombocytopenia or the platelet count. 7 of the patients, who relapsed after splenectomy had lipid-laden histiocytes in their spleens i.e. 58 % of the patients with relapse, compared with 10 patients with foamy histiocytosis and without relapse i.e. 19 % of the patients without relapse. At follow up, 3 of the 17 patients with splenic histiocytosis still had thrombocytopenia, compared with 1 of the 47 patients without foamy cells. Foamy histiocytosis of the spleen from a patient with chronic thrombocytopenia may indicate a dubious prognosis.     

Foamy histiocytosis of the spleen in patients with chronic thrombocytopenia.

In 17 of the 64 spleens removed from patients with chronic thrombocytopenia lipid-laden histiocytotes were demonstrated. No correlation was found between these pathological findings and the age of the patients at diagnosis or splenectomy, the duration before splenectomy of therapy with glucocorticoids, the period with thrombocytopenia or the platelet count. 7 of the patients, who relapsed after splenectomy had lipid-laden histiocytes in their spleens i.e. 58% of the patients with relapse, compared with 10 patients with foamy histiocytosis and without relapse i.e. 19% of the patients without relapse. At follow up, 3 of the 17 patients with splenic histiocytosis still had thrombocytopenia, compared with 1 of the 47 patients without foamy cells. Foamy histiocytosis of the spleen from a patient with chronic thrombocytopenia may indicate a dubious prognosis.     

Abnormal regional cerebral blood flow found by technetium-99m ethyl cysteinate dimer brain single photon emission computed tomography in systemic lupus erythematosus patients with normal brain MRI findings

In this study, technetium-^99m ethyl cysteinate dimer (^99mTc ECD) brain single photon emission computed tomography (SPECT) was used to detect regional cerebral blood flow (rCBF) of the brain in SLE patients with normal brain magnetic resonance imaging (MRI) findings. Twenty female SLE patients were enrolled in this study, divided into two groups. Group 1 consisted of 10 patients with neuropsychiatric manifestations. Group 2 consisted of 10 patients without neuropsychiatric manifestations. All patients had normal brain MRI findings. Another 10 SLE patients with abnormal MRI findings were included as group 3 for comparison. Meanwhile, 10 healthy female volunteers also underwent brain MRI and ^99mTc ECD brain SPECT for comparison. The scans revealed hypoperfusion lesions in 9/20 (45%) SLE patients, including 7/10 (70%) cases in group 1 and 2/10 (20%) cases in group 2. In contrast, all 10 patients (100%) in group 3 had abnormal ^99mTc ECD brain SPECT findings. The parietal lobes were the most commonly involved areas. We conclude that ^99mTc ECD brain SPECT is more sensitive for detecting rCBF changes than is brain MRI in detecting the brain anatomic changes, and may have a diagnostic value in lupus cerebral involvement. However, ^99mTc ECD brain SPECT may not be indicated for SLE patients with normal MRI and mild neuropsychiatric symptoms/signs, such headaches and dizziness. Received: 16 January 2002 / Accepted: 2 July 2002 Correspondence and offprint requests to: Dr Albert Kao, Departments of Nuclear Medicine and Medical Research, China Medical College Hospital, No. 2, Yuh-Der Road, Taichung 404, Taiwan. Tel: 886-4-22052121, ext. 3475; Fax: 886-4-22023442; E-mail: albertkaotw@yahoo.com.tw