Massive splenic infarction in Saudi patients with sickle cell anemia: a unique manifestation

Splenic infarcts are common in patients with sickle cell anemia (SCA), but these are usually small and repetitive, leading ultimately to autosplenectomy. Massive splenic infarcts on the other hand are extremely rare. This is a report of our experience with 8 (4 males and 4 females) cases of massive splenic infarction in patients with SCA. Their ages ranged from 16 to 36 years (mean 22 years). Three presented with left upper quadrant abdominal pain and massive splenic infarction on admission, while the other 5 developed massive splenic infarction while in hospital. In 5 the precipitating factors were high altitude, postoperative, postpartum, salmonella septicemia, and strenuous exercise in one each, while the remaining 3 had severe generalized vasoocclusive crises. Although both ultrasound and CT scan of the abdomen were of diagnostic value, we found CT scan more accurate in delineating the size of infarction. All our patients were managed conservatively with I.V. fluids, analgesia, and blood transfusion when necessary. Diagnostic aspiration under ultrasound guidance was necessary in two patients to differentiate between massive splenic infarction and splenic abscess. Two patients required splenectomy during the same admission because of suspicion of secondary infection and abscess formation, while a third patient had splenectomy 2 months after the attack because of persistent left upper quadrant abdominal pain. In all the 3 histology of the spleen showed congestive splenomegaly with massive infarction. All of our patients survived. Two patients subsequently developed autosplenectomy while the remaining 3 continue to have persistent but asymptomatic splenomegaly. Massive splenic infarction is a rare and unique complication of SCA in the Eastern Province of Saudi Arabia, and for early diagnosis and treatment, physicians caring for these patients should be aware of such a complication.     

Cytomegalovirus can make immune thrombocytopenic purpura refractory

Immune thrombocytopenic purpura (ITP) is characterized by decreased platelet numbers secondary to platelet destruction and reduced platelet production. Even if the ITP persists, it typically responds to 'ITP-specific' therapies, such as intravenous immunoglobulin, steroids, intravenous anti-D, and splenectomy. Several reports, including our previous study, have implicated cytomegalovirus (CMV) in the pathogenesis of infrequent cases of ITP that were not severe in nature. A recent study from China suggested that CMV is the aetiology of some cases of acute ITP of childhood and may require different treatment. We report two adult and two paediatric patients with refractory, severe, symptomatic thrombocytopenia, who were diagnosed with ITP and found to have active CMV infection. Their presentations included fever, transaminitis, neutropenia, and atypical lymphocytosis, but in particular, treatment-refractory, severe ITP. Treatment with steroids appeared to worsen the CMV-ITP. All four cases showed improvement in platelet counts within two weeks of starting ganciclovir and cytogam and tapering steroids. Based on the four patients described here, we believe that, in certain cases, CMV infection will result in symptomatic, severe, refractory ITP, which may be indistinguishable from typical ITP. Eradication of CMV with antiviral therapy improved the ITP in these cases.     

Recurrence of thrombocytopenia in patients splenectomized for idiopathic thrombocytopenic purpura

Summary The effects of splenectomy in the treatment of chronic idiopathic thrombocytopenic purpura (ITP) were analysed in 177 subjects (118 adults and 59 children) operated upon between two and 25 years earlier.In 145 patients, thrombocytopenia regressed immediately or within 2 months after the operation. Regression of thrombocytopenia later during follow up was observed in a further 14 cases.Recurrence of thrombocytopenia after good immediate result of splenectomy developed in 32 patients. In 13 cases the recurrence was transient but in the remaining 19 patients it was long-lasting. In these cases presence of accessory spleens was suggested by the absence of Howell-Jolly bodies in the peripheral blood smear and demonstrated by splenic scan in 16 cases, and confirmed in 13 out of 15 splenectomized patients. Resplenectomy caused in 12 patients permanent disappearance of thrombocytopenia.     

Massive intracranial bleeding requiring emergency splenectomy in a patient with CMV-associated thrombocytopenia.

We describe a previously healthy male patient, with severe immune thrombocytopenic purpura (ITP) following CMV infection which was refractory to steroids and intravenous immunoglobulin, who developed massive intracranial bleeding. Despite an extremely low platelet count (2x10(9)/liter) which was refractory to platelet transfusions, successful emergency splenectomy was performed, with rapid resolution of the thrombocytopenia. Bleeding complications are extremely rare in viral-associated ITP. Emergency splenectomy should be considered in the presence of life-threatening bleeding when other modalities fail to produce a rise in the platelet count. Infection with CMV should be ruled out in cases of severe, treatment-resistant ITP.     

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.     

A Study of Thrombocytopenia: New Histologic Criteria for the Differentiation of Idiopathic Thrombocytopenia and Thrombocytopenia Associated with Disseminated Lupus Erythematosus

Sixty-nine cases of thrombocytopenia in which splenectomy had been performed have been reviewed. New cytological criteria are described for thediagnosis of disseminated lupus erythematosus by the examination of thesplenic tissue.

Six cases of thrombocytopenia associated with the ingestion of drugs andeight with infectious diseases responded promptly and permanently to splenectomy. The thrombocytopenia associated with disseminated lupus erythematosus (in 16 cases) and idiopathic thrombocytopenic purpura (in 39 cases)had a much more variable response. Approximately three-fifths of patients ineach group had a remission sustained for at least 12 months following splenectomy. In general those patients who had thrombocytopenia for more thanone year before surgery were less likely to respond to splenectomy.

Submitted on November 19, 1966 Accepted on February 8, 1967     

Role of splenectomy in the management of hemophilic patients with human immunodeficiency virus-associated immunopathic thrombocytopenic purpura.

Immunopathic thrombocytopenic purpura (ITP) can be a life-threatening complication of human immunodeficiency virus (HIV) infection in patients with hemophilia and can seriously compromise quality of life if not managed effectively. We report here complete response to splenectomy in four severe hemophiliacs with HIV-associated ITP. All patients were symptomatic, had platelet counts less than 25,000/mm3, and had failed at least one non-surgical therapy prior to splenectomy. All patients tolerated surgery well and obtained an immediate and durable complete response. In addition to our experience, a review of the literature shows that splenectomy is well tolerated and provides the most effective long-term solution for hemophiliacs with HIV-ITP.     

MANAGEMENT OF SPLENECTOMY FAILURES IN CHRONIC IMMUNE THROMBOCYTOPENIC PURPURA: ROLE OF ACCESSORY SPLENECTOMY

Abstract Accessory splenic tissue was demonstrated in four of eight patients with chronic immune thrombocytopenic purpura investigated following post-splenectomy relapses. Time from initial splenectomy to relapse of thrombocytopenia ranged from immediately to eight years and post-splenectomy changes were present on the peripheral blood films of all patients at time of relapse. Three scanning techniques were employed to demonstrate and localise residual splenic tissue. Conventional ^99mTc scans were positive in three of the four patients whilst ^99mTc-heat damaged red cell scans and computerised tomographic scans were each positive in three out of three patients including the one patient in whom a conventional ^99mTc scan was negative. Histological confirmation of splenic tissue was obtained in all cases with the weights of the accessory spleens ranging from 0.6 g to 2 g. Two patients responded to accessory splenectomy and, without immunosuppressive therapy, have remained well with normal platelet counts for over four years. There was no correlation between length of initial remission and the response to removal of the accessory spleen. The presence of a functioning accessory spleen should be considered in all patients with chronic ITP who fail to respond to, or relapse following, initial splenectomy. (Aust NZ J Med 1986; 16: 695–698.)     

Usefulness of thrombopoietin in the diagnosis of peripheral thrombocytopenias.

BACKGROUND AND OBJECTIVE: Thrombocytopenia of peripheral origin is basically due to platelet destruction or splenic sequestration. Thrombopoietin (TPO) regulates platelet production stimulating megakaryocyte proliferation and maturation. The evaluation of TPO levels may be a useful tool in the diagnosis of thrombocytopenias of unknown origin. We tried to determine the value of TPO levels in some thrombocytopenias classically considered as peripheral. DESIGN AND METHODS: Serum TPO levels and platelet counts were measured in 32 thrombocytopenic patients with liver cirrhosis (LC) and 23 with chronic hepatitis C (CHC) viral infection, in 54 patients with a clinical and serological diagnosis of autoimmune thrombocytopenic purpura (AITP), and in 88 patients infected with the human immunodeficiency virus (HIV). RESULTS: Patients with LC, AITP and HIV had lower platelet counts than patients with CHC. The degree of thrombocytopenia did not, however, correlate with the TPO levels. HIV infected patients (246+/-304 pg/mL) and AITP patients (155+/-76 pg/mL) had higher TPO levels than controls (121+/-58 pg/mL). TPO levels in patients with CHC (125+/-40 pg/mL) did not differ from those in control subjects, but were slightly decreased in patients with LC (104+/-56 pg/mL). INTERPRETATION AND CONCLUSIONS: Reduced TPO production could be involved in the development of thrombocytopenia in LC patients, but not in patients with early stages of CHC viral infection. HIV and AITP patients had slightly raised levels of TPO. As TPO levels are normal or slightly increased in most peripheral thrombocytopenias, these data alone are not sufficient to distinguish the different types of peripheral thrombocytopenia. They may, however, be a useful tool for differentiating some central and peripheral thrombocytopenias.     

Human T-lymphotropic virus type I infection and idiopathic thrombocytopnic purpura

Human T-lymphotropic virus type I (HTLV-I) is the causative agent in adult T-cell leukemia and HTLV-I associated myelopathy. Some other diseases such as uveitis, chronic thyroiditis, Sj?gren syndrome, arthritis, acute myeloid leukemia and myelodysplastic syndrome may be also associated with HTLV-I. Several case reports have suggested the possible combination of idiopathic thrombocytopenic purpura (ITP) and HTLV-I infection. In these studies and from our current report, we found 17 patients (22.1%) with HTLV-I infection among 77 ITP patients. The prevalence of HTLV-I infection among ITP patients was higher than that of healthy volunteers (5 approximately 10%). The ITP patients with HTLV-I infection were older than the patients without HTLV-I infection, and the ITP patients with HTLV-I infection had poor response to prednisolone therapy. Among 17 ITP patients with HTLV-I infection, 9 patients received prednisolone therapy. Although most patients had transient increase of platelet counts, only two of them had partial responses (PR) at the last observation date. Five patients underwent splenectomy, and four of them had complete responses (CR) and the remaining patient had a (PR). Four patients received eradication of Helicobactor pylori (H. pylori) infection, and all patients had CRs. Therefore, the ITP patients with HTLV-I infection should receive eradication therapy in the case of H. pylori infection as the first step of therapy and the splenectomy should be considered, if there is no response to conventional therapy. Human immunodeficiency virus (HIV) causes thrombocytopenia in 10% of patients with active HIV disease. The etiologies of HIV thrombocytopenia are considered as follows, the escalated destruction of platelets by the immune system, damage to megakaryocytes by HIV infection and the inhibition of thrombopoiesis by some anti-viral drugs. In the case of ITP patients with HTLV-I infection, the main etiology may be the increased destruction of platelets by immune system. The proviral load and the integration pattern of HTLV-I should be examined to clarify the stage of HTLV-I infection. The possibility of infection of the megakaryocytes by HTLV-I should be also examined for etiological approach.     

Immune thrombocytopenic purpura in Hodgkin disease

Immune thrombocytopenic purpura is rarely seen in Hodgkin disease and the presence of platelet-associated antibody has not been previously reported in these patients. A patient with Hodgkin disease is described who developed a destructive thrombocytopenia demonstrated by shortened platelet survival. In conjunction with his thrombocytopenia, he had marked elevation of platelet-associated immunoglobulin G levels (nanograms IgG per 10(9) platelets: 15,187 prior to splenectomy and 71,130 and 81,900 after surgery). Mean values (+/-SD) of control subjects averaged 1,975 + 381 and four patients with Hodgkin disease and normal platelet counts had levels ranging from 1,581 to 4,011. We suggest that this patient had immune-mediated thrombocytopenia; whether the increase in platelet-associated immunoglobulin G was due to antiplatelet antibody or to adsorbed or phagocytosed immune complexes cannot be demonstrated by these studies. The platelet-associated immunoglobulin G test may be useful in evaluating these patients.     

Platelet survival and turnover: important factors in predicting response to splenectomy in immune thrombocytopenic purpura

Autologous indium-111 platelet sequestration and survival studies were performed on 59 immune thrombocytopenic purpura (ITP) patients, 21 of whom underwent splenectomy shortly thereafter. Sequestration patterns were primarily splenic in 46 patients, primarily hepatic in 6 patients, and both splenic and hepatic in 8 patients. The mean platelet survival ranged from 15 to 211 hr (normal, 180-220 hr), and mean platelet turnover (a measure of platelet production rate) varied from 99 platelets/microliters/hr to 7,585 platelets/microliters/hr (normal 1,200-1,600 platelets/microliters/hr). Among splenectomy patients, 13 had an excellent response, and 8 had a fair or poor response. Neither the pattern of platelet sequestration nor the quantity of platelet-associated IgG was useful in predicting response to splenectomy. There was, however, a striking correlation between platelet studies showing short survival/high turnover and subsequent excellent response to splenectomy. Conversely, patients with only moderately decreased survival and low turnover had an unpredictable response to splenectomy. This investigation demonstrates that ITP patients are a heterogeneous population and include a significant subset whose thrombocytopenia results primarily from decreased turnover. Platelet kinetic studies appear useful in predicting beneficial response to splenectomy.     

Splenic abscess and sickle cell disease

This is a report of our experience with 10 cases of splenic abscess in patients with sickle cell disease (SCD). All presented with fever and abdominal pain and were found to have a tender enlarged spleen. Two were found to have a ruptured spleen and five of them were septicemic on presentation. Although both ultrasound and CT-scan of the abdomen were of diagnostic value, we found CT-scan more accurate and reliable in the diagnosis of splenic abscess. Ultrasound and/or CT-scan should be used routinely in the evaluation of SCD patients who present with fever and abdominal pain, especially if they have a tender enlarged spleen. Diagnostic aspiration under CT-scan or ultrasound guidance should be used in doubtful cases to differentiate between splenic abscess and a large splenic infarct. All our patients were managed by peri operative antibiotics and splenectomy with no mortality. Salmonella was the commonest causative organism. Although CT-guided aspiration of splenic abscess is being advocated recently, we feel splenectomy should be the treatment of choice in patients with SCD as there is no point in preserving a non-functioning spleen that is present in the majority of patients. CT-guided aspiration may be employed as a temporary measure for those patients who are at high surgical risk with unilocular abscess. Am. J. Hematol. 58:100–104, 1998. © 1998 Wiley-Liss, Inc.     

Pregnancy in women with immune thrombocytopenic purpura

Thirty-six women with immune thrombocytopenic purpura were studied during 37 pregnancies, and maternal characteristics with predictive value for the fetal platelet count were determined. Nine neonates were thrombocytopenic, with a platelet count of less than 50 x 10(9)/L in eight. Four of these nine neonates delivered to a subgroup of 31 mothers were studied prospectively; the frequency of thrombocytopenia in neonates of women with immune thrombocytopenic purpura was thus 13%. Only two of these nine neonates presented with hemorrhagic syndromes (two, petechial purpura; one, intracranial bleeding). The frequency of neonatal thrombocytopenia was higher in mothers with deep thrombocytopenia and in those who had not responded to corticosteroid treatment following diagnosis. No prognostic value could be assigned to the other maternal characteristics studied, such as a history of splenectomy, maternal treatment at the time of delivery, or the presence of platelet autoantibodies evaluated either with the platelet immunofluorescence test or the platelet Western blot immunoassay.     

Partial splenic embolization.

Partial splenic embolization (PSE) is a non-surgical procedure developed to treat hypersplenism as a result of hepatic disease and thus avoid the disadvantages of splenectomy. A femoral artery approach is used for selective catheterization of the splenic artery. Generally, the catheter tip is placed as distally as possible in an intrasplenic artery. After an injection of antibiotics and steroids, embolization is achieved by injecting 2-mm gelatin sponge cubes suspended in a saline solution containing antibiotics. PSE can benefit patients with thrombocytopenia, esophagogastric varices, portal hypertensive gastropathy, encephalopathy, liver dysfunction, splenic aneurysm, and splenic trauma. The contraindications of PSE include secondary splenomegaly and hypersplenism in patients with terminal-stage underlying disease; pyrexia or severe infections are associated with a high risk of splenic abscess after PSE. Complications of PSE include daily intermittent fever, abdominal pain, nausea and vomiting, abdominal fullness, appetite loss, and postembolization syndrome. Decreased portal-vein flow and a rapid increase in the platelet count after excessive embolization may cause portal-vein or splenic-vein thrombosis.     

Immune thrombocytopenia in hemophiliacs infected with human immunodeficiency virus and their response to splenectomy

We studied five patients with hemophilia A in the age range of 18 to 64 years who were infected with human immunodeficiency virus and who developed immune thrombocytopenia. The clinical course of immune thrombocytopenia in relation to human immunodeficiency virus infection and the patients' responses to splenectomy and immune variables were determined. All five patients developed antibody to human immunodeficiency virus 6 to 60 months (median, 24 months) before the onset of thrombocytopenia, and two patients became human immunodeficiency virus antigenemic (one patient at the onset of immune thrombocytopenia and the other 60 months after the onset of immune thrombocytopenia [24 months after splenectomy]). All five patients had a strong platelet-associated immunoglobulin G and three patients also had a weak platelet-associated immunoglobulin M on their platelets. In four of five patients danazol therapy failed, and three patients required moderate doses of prednisone. Because of the progression of immune thrombocytopenia, four of the five patients underwent splenectomy with preoperative high-dose intravenous immune globulin. All four had an excellent immediate response to splenectomy, with a rise in platelet count to more than 300 x 10(9)/L and sustained remission during postsplenectomy follow-up of 6 to 45 months. There was no significant drop in CD4 and CD8 counts after splenectomy, and all four patients remained clinically well.     

Splenic thrombosis complicating pregnancy in patients with poor obstetrical outcome a report of four cases

Summary The association of pregnancy and splenic vein thrombosis is rare. The authors describe four patients who had acute painful splenomegaly in association with pregnancy. All patients had a slightly elevated platelet count in the nonpregnant state and had at least one spontaneous abortion. One patient had transient neurologic symptoms, and two others had a history of gastric ulcer. The splenic thrombosis occurred in the 9th (in two cases) and in the 13th week of gestation (in two cases), and 1 week after abortion (in two cases). Three patients under-went splenectomy and one refused it. All patients had persistent thrombocythemia after the splenectomy. In three patients the platelet count reversibly dropped at least 50% during pregnancy. Of their 19 pregnancies, one resulted in normal birth, one in preterm birth, two in therapeutic abortion, and 15 in spontaneous abortion. Data presented suggest that the latent form or early stage of a myeloproliferative disorder may contribute to both splenic and placental thrombosis.     

Portal vein thrombosis is a common complication following splenectomy in patients with malignant haematological diseases

BACKGROUND: Elective laparoscopic splenectomy (LS) is performed with increasing frequency rather than open splenectomy (OS) because of reduced morbidity. LS is feasible also in patients with haematological diseases with splenomegaly, a group that is subject to more postoperative complications, such as bleeding, infections and portal vein thrombosis (PVT). METHOD: We retrospectively reviewed the medical records of 69 patients splenectomised for haematological diseases during a 5-yr period at a single centre with the aim of comparing the results and complications after LS and OS. RESULTS: Thirty-nine patients underwent LS and 30 OS. The median durations of surgery were 138 and 115 min (ns) in the LS and OS groups respectively. Three conversions (7.7%) from laparoscopic surgery to open surgery were necessary because of bleeding and/or splenomegaly. Thromboembolic complications occurred in totally seven of 69 patients. PVT was diagnosed in five of 37 (13.5%) patients with haematological malignancies (three with indolent lymphoma and two with myeloproliferative disease), one after LS and four after OS. All patients with PVT had splenomegaly and had received thromboembolic prophylaxis with low-molecular-weight heparin of short duration. Two patients were diagnosed with deep vein thromboses in the lower leg. Both had idiopathic thrombocytopenic purpura (ITP) and LS. CONCLUSIONS: Patients with malignant haematological diseases and splenomegaly seem to have a high risk of developing PVT after splenectomy why careful observation and prolonged thromboprophylaxis is recommended for these patients. Ultrasonography or computerised tomography should be considered in all patients with abdominal symptoms after splenectomy.     

Immune thrombocytopenic purpura: autoimmune or immune complex disease?

To assess the pathogenic role of circulating immune complexes (CIC) in idiopathic thrombocytopenic purpura (ITP), 39 patients with ITP were compared to 17 patients with other forms of thrombocytopenia (hypersplenism (N = 12), impaired thrombopoiesis (3), thrombocytopenia of unknown origin (2)) and six nonthrombocytopenic subjects. In all patients, platelet mean life span (MLS), platelet associated IgG (PAIgG), as well as circulating anti-platelet antibodies and C1q binding activities were determined. In most cases, immune complex solubilization capacity (ICSC) and immune complex precipitation inhibition capacity (ICPIC) of sera were also assessed. All patients with ITP had a reduced platelet MLS, but PAIgG was elevated in only 16 out of 24 patients with chronic ITP, in six out of 10 patients with acute ITP and in four out of five patients with secondary ITP. In the group of patients with thrombocytopenia due to splenomegaly, seven out of 12 patients had elevated PAIgG while the platelet MLS was only slightly reduced. Of the 39 patients with ITP only one with secondary ITP had C1q binding material in his serum, as opposed to six out of 12 thrombocytopenic patients with splenomegaly. Whereas only three patients with ITP had abnormal immune-complex modulating capacities, such deviations were found in seven out of 12 patients with thrombocytopenia due to splenomegaly. We conclude that our data render the role of CIC in the pathogenesis of ITP very questionable.     

Successful management of thrombocytopenia by partial splenic embolization in patients with advanced gastric cancer and invasion of the splenic vein: Case reports

Rationale:Hypersplenism causes thrombocytopenia, which may lead to the reduction or discontinuation of chemotherapy. Partial splenic embolization (PSE) is an effective treatment for thrombocytopenia associated with hypersplenism. However, there have been no reports of patients with gastric cancer who have resumed and continued chemotherapy after PSE for splenic hypersplenism associated with tumor infiltration.Here, we report two cases in which we performed PSE for hypersplenism associated with gastric cancer that had invaded the splenic vein. Chemotherapy was continued in both cases.Patient concerns:Both patients developed thrombocytopenia with splenomegaly due to advanced gastric cancer that required discontinuation of chemotherapy.Diagnosis:Upper gastrointestinal endoscopy and computed tomography showed advanced gastric cancer with invasion of the splenic vein and splenomegaly. Both patients developed thrombocytopenia.Interventions:Patients were treated with PSE.Outcomes:PSE produced an increase in thrombocyte count, and chemotherapy could be resumed.Lessons:PSE seems to be a useful treatment for thrombocytopenia with splenomegaly associated with advanced gastric cancer and may allow continuation of chemotherapy.