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PubMed1996

Thrombo-embolic disease after splenectomy for hereditary stomatocytosis.

Nine cases of hereditary stomatocytosis (HSt) are presented which show documented thrombotic complications after splenectomy. In three cases, patients became severely ill with pulmonary hypertension and a fourth developed portal hypertension. One unsplenectomized affected adult relative had suspected but unconfirmed thrombotic pathology; the six other affected unsplenectomized adults did not. Since splenectomy is of only limited therapeutic benefit in stomatocytosis, it should not be performed without careful consideration. A tendency to iron overload, even without hypertransfusion and irrespective of splenectomy, is evident in many of these patients.

Adult↗
PubMed2000

Splenectomy in haemophagocytic lymphohistiocytosis: report of histopathological changes with CD19+ B-cell depletion and therapeutic results.

The pathogenesis of haemophagocytic lymphohistiocytosis (HLH) in children without a known familial pattern of inheritance is often difficult to establish. Splenic enlargement, one of the main clinical findings in this disorder, has led to the use of splenectomy for uncontrollable coagulopathy, persistent cytopenia or both. This procedure is also thought to be a useful tool in making a differential diagnosis in cases of the immunochemotherapy-resistant HLH. We report here five cases of splenectomized childhood HLH, in which subsets of mononuclear spleen cells were analysed either by flow cytometry or immunohistochemistry, and the results were compared with those from cases of hereditary spherocytosis (controls). There was a statistically significant depletion of CD19+ B cells in the HLH cases (3.8 +/- 3.2% vs. 52.6 +/- 4.5%, P < 0. 0001) associated with an increase of T cells in three cases and of natural killer cells in another. The histopathological findings included atrophic white pulps, B-cell depletion with fibrosis and haemosiderosis in all five cases. Despite temporary therapeutic benefits, three of the HLH patients had a rapidly deteriorating post-splenectomy course and all three eventually died. These results demonstrate striking depletion of B cells in the enlarged spleens of children with HLH, which may be an intrinsic feature of HLH pathogenesis. Further study is needed to establish the therapeutic value of splenectomy in this disease.

Antigens, CD19↗
PubMed2002

Prolonged inhibition of von Willebrand factor-cleaving protease after splenectomy in a 22-year-old patient with acute and plasma refractory thrombotic thrombocytopenic purpura.

We report a 22-year-old woman with acute, plasma refractory thrombotic thrombocytopenic purpura (TTP) in whom splenectomy led to consistent stabilization of platelet counts, but who showed complete inhibition of vonWillebrand factor-cleaving protease (VWF-cp) after 6 months of follow up. Persistent protease deficiency and resolved clinical and haematological TTP symptoms resulted in the transient appearance of unusually large VWF multimers in the patient plasma. As low but significant protease activity (10%) was first detectable as late as 9 months after splenectomy, we conclude tentatively that, at least in a subgroup of patients with acquired TTP, the beneficial effect of splenectomy is not exclusively due to the removal of splenic B lymphocytes producing an inhibitor of VWF-cp.

ADAM Proteins↗
PubMed2003

Long-term follow-up of chronic autoimmune thrombocytopenic purpura refractory to splenectomy: a prospective analysis.

Splenectomy remains the most effective treatment of chronic autoimmune idiopathic thrombocytopenia (ITP) (i.e. of > 6 months duration). Treatment of patients refractory to splenectomy (with absence of response or relapse after initial response) is difficult, and their long-term outcome is not well known. Over a 10-year period, 183 patients with chronic ITP were splenectomized including 158 adults and 25 children (</= 15 years). Forty-seven of them, who were refractory to this treatment, were prospectively followed up for 5-15 years (median 7.5 years). Twelve of them, with moderate thrombocytopenia, remained untreated, and 35 were treated by a median of two regimens (range 1--6), to which 27 responded. Thirty-six (77%) of the refractory cases reached platelet counts durably > 100 x 10(9)/l, nine of them without treatment and 27 of them with low-dose steroids or azathioprine; six (13%) remained moderately thrombocytopenic (35 x 10(9)/l to 100 x 10(9)/l platelets); the last five patients, without response to any treatment (up to six regimens), remained severely thrombocytopenic (platelets < 20 x 10(9)/l), and three of them died from bleeding. Twenty-seven (57%) of the 47 refractory cases required at least one hospitalization, in the majority of cases for intravenous immunoglobulin (IVIg) infusions. Seven of the refractory cases occurred in children. Six of them subsequently reached platelet counts > 100 x 10(9)/l, but one died from bleeding. Our findings confirm the overall favourable long-term prognosis of chronic ITP refractory to splenectomy.

Adult↗
PubMed2002

Splenectomy in a case of splenic vein thrombosis unmasks essential thrombocythemia.

We report a patient with splenic vein thrombosis (SVT) in whom splenectomy resulted in the unmasking of essential thrombocythemia (ET). He had portal hypertension with haematemesis, resulting in anaemia requiring repeated blood transfusions. Investigations revealed SVT. Following splenectomy, he suffered a transient ischaemic attack episode, associated with persistent thrombocytosis (> 2000 x 10(9)/l). Other myeloproliferative disorders were excluded and a diagnosis of ET was established. He responded to hydroxyurea but, due to financial constraints, he discontinued treatment and subsequently relapsed. The association of ET with SVT is rare and the diagnosis of ET was missed initially as the platelet count was normal prior to splenectomy.

Adult↗
PubMed2000

Laparoscopic versus open splenectomy in children.

BACKGROUND: The authors have reviewed their initial experience with laparoscopic splenectomy (LS) to identify the indications, success rate, and complications associated with this procedure compared with a series of children undergoing open splenectomy (OS) during the same time period. METHODS: The records of 51 children who underwent splenectomy from 1993 through 1998 were reviewed retrospectively. RESULTS: Thirty-five patients aged 1 to 17 years (mean, 9.4 years) underwent LS for the following indications: ITP (n = 20), sickle cell disease or thalassemia (n = 6), hereditary spherocytosis (n = 5), other hematologic disorders (n = 4). Seventeen patients aged 2 to 17 years (mean, 11.8 years) underwent OS during the same time period for ITP (n = 4), sickle cell disease or thalassemia (n = 4), hereditary spherocytosis (n = 5), and other indications (n = 4). Concomitant cholecystectomy was performed in 4 of 35 LS and 4 of 17 OS. Accessory spleens were identified in 10 of 35 LS and 2 of 17 OS cases. Eleven spleens were enlarged in the LS group, and 8 were enlarged in the OS group. One LS required conversion to an open procedure because the spleen did not fit in the bag. No other cases were converted. Median estimated blood loss was 50 mL for both the LS and OS groups. The only intraoperative complication in the LS group was a splenic capsular tear, which had no effect on the successful laparoscopic removal of the spleen. No patient in either group required a blood transfusion. The LS patients had a shorter length of hospital stay (1.8 +/- 1 versus 4.0 +/- 1 day, P = .0001). Total hospital charges were not significantly different. Follow-up ranged from 6 to 40 months. One LS patient died 47 days postoperatively from unrelated causes. Two LS patients had recurrent ITP; accessory spleens were found in one and resected laparoscopically. CONCLUSION: LS in children can be performed safely with a low conversion rate (2.9%) and is associated with a shorter hospital stay and comparable total hospital cost when compared with OS.

Adolescent↗
PubMed2002

Role of splenectomy in immune (idiopathic) thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura (ITP) is an immune disorder, which causes an acute or chronic thrombocytopenia, and may result in potentially life-threatening hemorrhage. Splenectomy is one of the treatment options that needs to be weighed in the treatment of ITP, particularly in cases that have shown response failure to medical modalities such as prednisone, i.v.Ig, or anti-D globulin therapy. Although most studies demonstrate good early response following splenectomy, the long-term outcome is less favorable. Furthermore, other negative factors, such as rendering the patient ineligible for anti-D globulin or oral tolerance therapy and vulnerable to possible life-threatening sepsis, must be weighed prior to splenectomy.

Disease Management↗
PubMed1977

[Primary and secondary exploratory laparotomy and splenectomy in Hodgkin's disease (author's transl)].

Exploratory laparotomy with splenectomy was performed on 275 patients with hisologically confirmed Hodgkin's disease. In 188 patients the laparotomy was a primary one to determine more precisely the state of the disease. A secondary laparotomy was performed in 87 patients 1-12 years after diagnosis and radiotherapy. In 17.5% of patients the state had to be revised after laparotomy with splenectomy. In 38 the disease had further progressed, while in ten it had slighty regressed. In four cases clinical stage I proved to be stage III. Even prognostically more favourable forms may have progressed at first diagnosis. There was no correlation between B-symptoms and histological type, but there was between B-symptoms and spread of the disease. Calculated spleen weight provided no clue as to spleen involvement. There was no clear relationship between spleen involvement and histological subclassification. Risk-effect analysis indicate that laparotomy with splenectomy was useful because it makes optimal treatment possible.

Adult↗
PubMed1978

[Scintigraphic evidence of changes in liver shape following splenectomy (author's transl)].

It has been suggested in the literature that, following splenectomy, the shape and position of the liver become altered and that this may lead to errors in the interpretation of the liver scans. For this reason, liver scans before and after splenectomy were compared in 16 patients with Hodgkin's disease. No changes in shape or position of the liver were found which could be related to the splenectomy. However, the clinical features and treatment must be known to ensure correct interpretation.

Hepatomegaly↗
PubMed2004

[Accessory spleens--diagnostic and therapeutic problem of the laparoscopic splenectomy in idiopathic thrombocytopenic purpura patients].

The preoperative detection of accessory spleens (AS) is essential in ITP patients. The aim of this study was to evaluate the reasons of failure and long-term results of laparoscopic splenectomy (LS) in patients with idiopathic thrombocytopenic purpura (ITP). Thirty-four ITP patients (27 females and 7 males) underwent LS between June 1998 and January 2001. Computed Tomography (CT) and sonography (US) were performed preoperatively to evaluate the size of the spleen and to detect the presence of possible accessory spleens which were found in two cases. AS were seen during laparoscopy in three cases. During follow-up (median time = 23 months), in three patients a low platelet count was seen after 5 months, 1.5 and 1.8 years pop. In all these cases scintigraphy was performed, which revealed in one case the residual accessory spleen. In the two other patients, inspite of thrombocytopenia no residual spleens were found. The authors conclude that laparoscopic splenectomy is a safe and effective procedure in patients with ITP. The problem of accessory spleens can be managed by careful videoscopic examination of the abdominal cavity during splenectomy. The use of the preoperative imaging techniques for detection of accessory spleens is limited by the insufficient sensitivity of the examination.

Adolescent↗
PubMed1982

Mitral valve replacement and splenectomy in a patient with chronic idiopathic thrombocytopenic purpura.

We report the management of a patient with chronic idiopathic thrombocytopenic purpura and mitral valve disease. Although a two-stage approach was planned (splenectomy followed by mitral valve replacement one month later), the patient developed medically-resistant heart failure, and splenectomy plus mitral valve replacement were performed during the same operation. The platelet count at operation was 20,000/mm3. Platelet transfusion, used at the end of cardiopulmonary bypass, was considered no longer necessary in the postoperative period, as the platelet count quickly increased after the first postoperative day. The postoperative course was uneventful. Though we believe the two-stage surgical approach is preferable, our case shows that open-heart operations and splenectomy can be successfully performed simultaneously in patients with idiopathic thrombocytopenic purpura.

Blood Transfusion↗
PubMed1996

[Immune response to a single dose of a novel kind of hepatitis A vaccine following splenectomy].

BASIC PROBLEM AND OBJECTIVE: The immune response to parenteral antigens is reduced in persons without spleen. The seroconversion rate was measured after application of a new type of vaccine against hepatitis A (immunopotentiating reconstituted influenza virus virosome [IRIV]) in patients who had undergone a splenectomy after trauma. PATIENTS AND METHODS: 26 patients (23 men and 3 women, mean age 34.9 +/- 9.7 [25-65] years) with anti-hepatitis A virus (HAV) antibody titres < 20 mIU/ml (maximally 17 mIU/ml) were given a single dose of 0.5 ml of the IRIV hepatitis A vaccine 1 to 14 (mean 9.4) years after splenectomy. Immediately after the immunisation and 14 and 28 days afterwards anti-HAV titres were determined. A titre rise to < 20 mIU/ml was counted as seroconversion. At the first and last titre measurement immunoglobulins, neopterin and beta-microglobulin levels were also measured as additional markers. RESULTS: The seroconversion rate was 69.2% (18/26) after 14 days (geometric titre mean: 39 mIU/ml) and rose to 88.5% (23/26) after 28 days (geometric titre mean 74 mIU/ml). Seroconversion occurred in the three nonresponders after a second dose of the vaccine. All measurements of the immunological markers were within normal limits. CONCLUSION: A single dose of a new type of vaccine against hepatitis A confers adequate protection even in those persons who had a splenectomy.

Adult↗
PubMedDate not supplied

[Splenectomy and reimplantation of splenic tissue in children].

161 splenectomies are presented with a morbidity of 14.8% and a total mortality rate of 4.3%. The lethality rate due to severe infections (-3.9%), however, was high and mostly caused by an overwhelming postsplenectomy sepsis, pneumonia or meningitis. 61.5% of the infections were caused by pneumococcus. To drop that high lethality rate after splenectomy we replanted splenic tissue in seven patients. Scintigraphic investigations showed in all cases well vascularised splenic tissue. The immunoglobulins were in a normal range. Howell-Jolly bodies, however, did not disappear in all the patients. From our study as well as from the literature one has to conclude that replanted splenic tissue requires immunologic activity but a reduced capacity for phagocytosis. Therefore, in cases of traumatic injury to the spleen it is necessary to try to preserve as much of the organ as possible either by infrared coagulation, gluing with human fibrinogen or partial splenectomy. If these procedures are impossible a replantation of--50% of the original splenic tissue should be done and several thin homogenised particles replanted intra- or retroperitoneally. Out of that a preoperative vaccination against pneumococcal infection is necessary as well as a postoperative penicillin prophylaxis for about three years.

Child↗
PubMed1981

Complications after elective splenectomy in children.

Elective splenectomies performed in children aged 0-14 during the period 1968 to 1977 have been reviewed. One hundred and twelve children were splenectomized during the period studied. The indication for splenectomy was usually a haematological disorder e.g. hereditary spherocytosis or idiopathic thrombocytopenia. As splenectomized children run the risk of developing overwhelming sepsis, inquiries have been sent to all children. Two cases of nonfatal pneumococcal septicaemia are reported. Although the incidence of septicaemia is low, spleensaving operations are to be preferred where possible. When splenectomy is performed, pneumococcal antibodies should be determined. If a low titre is found, polyvalent pneumococcal vaccine should be used.

Adolescent↗
PubMed1995

Results of splenectomy performed on a group of 91 children.

In the period 1971-1990 91 children underwent splenectomies in the University Hospital of Nijmegen. The most important indications are hereditary spherocytosis, Hodgkin's disease and very severe immune thrombocytopenic purpura (ITP). Splenectomy after a traumatic rupture of the spleen has become less frequent: from 20% in 1971-1980 to 4% in 1981-1990. Short-term complications included thrombocytosis (84%), fever without an obvious cause (46%), which is quite regularly seen in patients suffering from Hodgkin's disease (48%), and infections of the respiratory tract in 10% of the patients. The platelet count shows a steady increase in the first nine post-operative days. No thromboembolic complications were seen. Based upon the literature there seems to be no reason at this moment for anti-platelet aggregation therapy when platelet counts are below 1000 x 10(9)/l. More information about long-term complications was obtained through a questionnaire completed by general practitioners. The morbidity through overwhelming post splenectomy infection (OPSI) is 3.8% (3/79), the mortality of OPSI is 2.5% (2/79). Underlying diseases, especially those which involve the immunological system as auto immune haemolytic anemia (AIHA), seem to play an important role in the possible development of OPSI (morbidity 2/11, 18%).

Adolescent↗
PubMed1985

[The role of splenectomy in the treatment of lymphomas of low malignancy].

The effect of splenectomy was studied in 30 patients with non-Hodgkin lymphoma of low malignancy. Severe bacterial infections occurred in 9 patients. Follow-up of the total group of 72 patients-years revealed one infection requiring hospitalization per eight patient-years. With chronic lymphatic leukaemia there was no difference in the incidence of severe infection between splenectomized and non-splenectomized patients. In 12 patients the splenectomy produced favourable results in that in some (8) cytostatic treatment was no longer required, while in others (4) the dose of cytostatic drugs could be reduced after several months without any medication. Survival rate was not influenced by splenectomy.

Adult↗
PubMed1986

Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis.

To determine how various inheritance patterns and responses to splenectomy relate to erythrocyte spectrin deficiencies in hereditary spherocytosis, we measured the spectrin content of erythrocytes by radioimmunoassay in 33 patients with this disease. Patients with the dominant form of hereditary spherocytosis generally had mild anemia, with spectrin at 63 to 81 percent of normal levels. Patients with the nondominant form of the disease had anemia ranging from severe to mild, with corresponding spectrin levels of 30 to 74 percent; their siblings were affected similarly. Distantly related homozygotes had different clinical severities with correspondingly different spectrin levels. The parents and offspring of patients with the nondominant form were clinically normal but consistently had subtle erythrocyte abnormalities. Spectrin levels in all patients were inversely related to osmotic fragility (P less than 0.0001), and they were also correlated with the clinical response to splenectomy: patients with spectrin levels above 70 percent achieved normal blood counts, those with levels of 40 to 70 percent had compensated hemolysis, and those with levels below 40 percent improved but remained anemic (P less than 0.0001). We conclude that the inheritance pattern and response to splenectomy in hereditary spherocytosis reflect erythrocyte spectrin deficiencies as determined by radioimmunoassay.

Anemia, Hemolytic, Congenital↗
PubMed2002

A prospective randomized study comparing D2 total gastrectomy versus D2 total gastrectomy plus splenectomy in 187 patients with gastric carcinoma.

BACKGROUND: Classic surgical treatment of upper third gastric carcinoma is based on an extended total gastrectomy, including splenectomy. The purpose of this study was to perform a prospective randomized clinical trial comparing the early and late results of total gastrectomy (TG) versus total gastrectomy plus splenectomy (TGS). METHODS: One hundred eighty-seven patients with gastric carcinoma were included. In all patients a D2 total gastrectomy was performed. During surgery they were randomized to 1 of 2 operative options. They were monitored to their death or to 5 years later if they were alive. RESULTS: Operative mortality was similar after both operations (3% after TG and 4% after TGS). Septic complications after surgery were higher after TGS compared with TG (P <.04). Five-year survival rates were not statistically different between groups or in subset analysis according to stage of disease. CONCLUSIONS: On the basis of the results of the present prospective randomized trial, splenectomy is not necessary in early stages of disease. A low operative mortality rate (less than 3%) must be achieved to obtain good long-term results.

Adult↗