Splenic irradiation in myelofibrosis with myeloid metaplasia: a review.

Morbidity from myeloid metaplasia and myelofibrosis arises from progressive anemia and abdominal discomfort related to massive splenomegaly, which may be associated with hypercatabolic symptoms. To date, no therapy, other than allogeneic bone marrow transplantation, has been shown to cure or to prolong the survival of these patients. Thus, current management strategies are palliative and include red cell transfusional support and androgen therapy for anemia; chemotherapeutic agents for control of thrombocytosis, leukocytosis, and hypermetabolic symptoms; and splenectomy or splenic irradiation for symptomatic splenomegaly. The major indication for splenic irradiation is left upper quadrant discomfort related to massive splenomegaly, usually in patients for whom splenectomy is contraindicated or has been declined. In most patients, it provides relief from abdominal pain and a moderate reduction in splenic size. Although responses are transient, some patients may experience prolonged relief. Splenic irradiation can result in prolonged myelosuppression in certain patients. This calls for cautious dosing, because individual sensitivity is variable and cannot be predicted. The use of splenic irradiation does not preclude subsequent splenectomy; however, the increased risk of postoperative hemorrhage should discourage consideration of splenic irradiation as an alternative or a temporizing measure before splenectomy when indicated.     

Cavitating splenic infarction

A patient with chronic myelogenous leukemia and massive splenomegaly is described in whom an acute abdominal catastrophe developed shortly after the initiation of chemotherapy. Sudden total infarction of the spleen occurred with occlusion of the splenic artery and vein; later a clostridial abscess formed within the necrotic spleen tissue producing a unique combination of clinical, roentgenographic and isotope scan findings. Possible pathophysiologic mechanisms for this process are discussed together with the clinical diagnostic and bacteriologie features.     

Bacteroides (Parabacteroides) distasonis splenic abscess in a sickle cell patient

Splenic abscess is not an uncommon complication of patients with sickle-cell disease. Here we describe an 18 year-old boy with sickle cell disease and left upper quadrant abdominal pain. Computerized axial tomography revealed left sided free flowing pleural effusion and splenomegaly with liquefaction and possible gas formation. The splenic fluid grew an unusual organism known as Bacteroides distasonis. The patient received antimicrobial therapy and underwent a splenectomy with full recovery. The spleen was cystically infarcted and measured 22 x 16 x 5 cm. The capsule was thickened and covered by fibrinous exudate. Histopathologic examination of the spleen showed complete necrosis with reparative fibrosis. This case presents an unusual cause of splenic abscess due to Bacteroides distasonis with a subacute to chronic course. The presence of fever and left sided pleuritic chest pain in patients with sickle cell disease should raise the suspicion of splenic abscess.     

Non hypoxia-related splenic infarct in a patient with sickle cell trait and infectious mononucleosis

Splenic infarction in patients with sickle cell trait is usually related to hypoxic conditions, while non-hypoxia-related infarcts are extremely rare. We report on a case of a 17-year-old male patient, living at sea level, who developed a severe left upper quadrant abdominal pain during the course of a febrile episode. On physical examination he had a mildly palpable but extremely painful spleen. A spleen scan revealed 2 areas of impaired radionucleide distribution. Hepatic enzymes were moderately increased and the IgM anti-EBV antibodies positive. Hemoglobin electrophoresis revealed the presence of 42% of hemoglobin S. A probable diagnosis of splenic infarction was established in a patient with sickle cell trait, during the course of infectious mononucleosis. The patient was treated symptomatically. The conditions of splenic congestion induced by the EBV infection and the high-grade fever may have contributed to splenic sequestration and subsequent infarcts.     

Rectus Sheath Hematoma Mimicking Acute Splenic Disease

In patients with hematological malignancies and splenomegaly, acute abdominal pain in the left upper quadrant is highly suspicious of splenic disease (i.e., hematoma, infarction, or rupture). We report the case of a patient with chronic myelogenous leukemia and splenomegaly who presented an unusual abdominal condition causing pain in the left upper quadrant, with a clinical presentation mimicking acute splenic disease. The diagnostic dilemma was resolved by ultrasonography, demonstrating a rectus sheath hematoma.     

First report of reversal of organ dysfunction in sickle cell anemia by the use of hydroxyurea: splenic regeneration

Much of the morbidity associated with sickle cell anemia (SCA) is due to ongoing infarction resulting in organ dysfunction. Because the spleen is often the first organ damaged in this illness, there is a significant impairment of the immune system in these patients. Hydroxyurea (HU) has been shown to increase fetal hemoglobin (HbF) and decrease painful episodes in patients with this disease. It is unclear whether HU can prevent organ damage. We treated two SCA patients with HU for several years and found evidence of reversal of previously documented splenic dysfunction. Patient no. 1 was treated for 30 months with an increase in HbF to 30%. HU was stopped because of cytopenia. She developed left upper quadrant pain. A splenectomy was performed due to the possibility of splenic abscesses. A pathologic review found no evidence of infection and an enlarged spleen that showed active germinal centers. Patient no. 2 was treated for 24 months with HU before developing splenomegaly. His HbF levels were 25% to 30%, his pit counts averaged 2%, and his liver spleen scans showed uptake. These two cases show that chronic HU therapy may reverse splenic dysfunction in certain patients and suggest that this drug may have efficacy beyond the elimination of pain in SCA.     

Spontaneous splenic rupture during induction chemotherapy for acute promyelocytic leukemia successfully treated with splenectomy

We report a case of acute promyelocytic leukemia who suffered spontaneous splenic rupture with massive hemoperitoneum while receiving intensive induction chemotherapy. Emergency computed tomography of the abdomen helped in the diagnosis of intra-abdominal bleeding. The patient was successfully treated with immediate splenectomy and made an uneventful postoperative recovery. Ten days after surgery chemotherapy could be resumed and complete remission was achieved. Although spontaneous splenic rupture is a rare complication of hematologic malignancies, this diagnosis should be considered in all patients with leukemia who develop acute abdominal pain with hypotension, even in the absence of splenomegaly.     

Radiographic appearance of diffuse splenic hemangiomatosis

The evaluation of splenomegaly in a patient with a consumptive coagulopathy included a liver-spleen scan, an abdominal sonogram, and an abdominal computed tomographic scan, which demonstrated an enlarged spleen with a diffuse parenchymal textural abnormality. A diffuse hemangiomatous pattern was present on splenic angiography. A splenectomy was performed and splenic hemangiomatosis was confirmed. The radiographic findings in this rare entity are presented and discussed.     

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.     

Splenic abscess disclosing endocarditis

A 54 year old man, hospitalised for thoraco-abdominal pain resulting from a septicemia which gives positive hemocultures for streptococcus D Bovis, is diagnosed to have a splenic abscess which will require splenectomy. At the same time, an endocarditis develops and gets worse, with auriculo-ventricular blockade and, especially, major aortic insufficiency, which is the cause of death by a brutal and massive pulmonary oedema. In the progression of an endocarditis, the occurrence of a splenic abscess, primary localisation of the initial septicemia or the secondary of an arterial septic embolism, is a rare contingency compared to the frequency of splenomegaly or splenic infarction: less than 2 percent of the cases in the literature. This very atypical and exceptional case serves as a reminder, on the one hand, of the diagnostic inadequacy of echocardiography which cannot visualise vegetation in the course of progressive endocarditis, and, on the other, of the prognostic importance of auriculoventricular blockade in septal and aortic endocardial lesions.     

脾脓肿19例临床资料分析

  目的 分析脾脓肿的临床诊治情况,为其诊疗提供参考。方法 回顾1991年1月—2012年3月北京协和医院19例脾脓肿患者的诊疗情况,分析其一般情况、基础疾病、临床表现、影像学特征、病原学依据、治疗方式、转归等临床资料。结果 19例脾脓肿患者从发病到就诊北京协和医院的中位时间为29 d,9例治愈,8例好转,2例死亡。大多数患者具有脾脓肿的危险因素,如肿瘤、糖尿病和免疫抑制状态等。脾脓肿临床表现不特异,19例患者脾脏均存在影像学变化;最多见的3种临床症状为发热（18例）、畏寒（12例）、寒战（11例）;最多见的3种体征是腹部压痛（9例）、左季肋区叩痛（7例）、脾大（4例）;病原菌培养结果显示最多见者为革兰阴性杆菌（9例）,革兰阳性球菌（8例）,真菌（4例）,7例患者存在2种或2种以上病原菌感染。结论 脾脓肿患者临床表现特异性不高。对于具有危险因素者,应进行相关临床检查,避免漏诊。结合超声等影像学检查,尽早诊断并开始经验性抗感染治疗;及时留取脓液等寻找病原学资料;根据患者情况,个体化选择治疗方案。        

Splenectomy in patients with AIDS

To establish the indications for splenectomy in patients with human immunodeficiency virus (HIV) infection we retrospectively analyzed 12 patients who underwent splenectomy. Patients with HIV infection who had immune thrombocytopenic purpura (ITP) were excluded as they had no splenomegaly and a definite indication for splenectomy exists in some of these patients. All 12 patients were anemic; 6 were thrombocytopenic and 6 leukopenic. All patients had splenomegaly and all were febrile. At surgery 3 patients were found to have Mycobacterium avium intracellulare (MAI) infection; 2 had splenic abscess due to Salmonella group D; 1 each had cytomegalovirus (CMV) splenitis and localized Kaposi's sarcoma (KS) of the spleen. No definite histopathologic diagnosis could be made in five patients, all of whom had evidence of extramedullary hematopoiesis. The degree of splenic enlargement did not correlate with the outcome. Both clinical and hematologic improvements were achieved in patients with splenic abscess and in patients who had splenomegaly, anemia, and thrombocytopenia. The presence of either of these findings constitutes an indication for splenectomy. Anemia and/or leukopenia without thrombocytopenia failed to improve; the presence of MAI and active CMV infection also resulted in failure. The presence of either of these conditions may be considered a contraindication to splenectomy.     

Splenic hydatidosis: a rare differential diagnosis in a cystic lesion of the spleen

Cystic tumours of the spleen are generally rare, and a parasitic origin is relatively unlikely. The present case report shows, however that when a splenic cyst is found, the differential diagnosis must always consider the possibility of echinococcosis. We report the case of a patient suffering from a cystic lesion of the spleen where surgery and histopathology yielded the diagnosis of splenic echinococcosis. Abdominal pain in the left upper quadrant and splenomegaly detected by simple abdominal radiology are the most commonly found indicators for this disease. The treatment should be surgical, attempting to preserve as much splenic tissue as possible, although conservative treatment is frequently unfeasable due to massive involvement of the spleen. Although rare, splenic hydatidosis should be included in the differential diagnosis when a cystic splenic lesion is identified with sonography or CT scan.     

Splenic torsion

Summary A 28-year-old woman who presented with acute abdominal pain and splenomegaly was found to have little functioning splenic tissue on liver-spleen scan but a large homogeneous spleen by computerized axial tomography. Laparotomy revealed torsion of the splenic pedicle and extensive splenic infarction. The presentation, pathophysiology, and diagnostic difficulties of this case are discussed. Splenic torsion should be considered in the differential diagnosis of painful splenomegaly.     

Splenic abscesses: a review of 20 cases

Splenic abscesses are rare. We present 20 new cases diagnosed in Denmark from 1982 to 1987. Five cases were diagnosed at autopsy. 10 were initially treated with splenectomy, 1 died. Five were treated with drainage and antibiotics resulting in 1 treatment failure who survived after splenectomy, and 1 death. The incidence of splenic abscesses in Denmark was 0.056% per 1,000 somatic hospital discharges per year or for the 5 cases discovered at autopsy 0.0049% per year of all hospital deaths. Splenic abscess should be suspected in the febrile patient with left upper quadrant tenderness and splenomegaly and the diagnosis confirmed by ultrasonography or computerized tomography. Treatment of choice is still splenectomy and antibiotics although favorable results have been achieved with drainage procedures in combination with antibiotics.     

A case of splenic pseudocyst difficult to differentiate from splenic lymphangioma]

Splenic pseudocyst is a rare complication of abdominal trauma. Although it is rare, splenic pseudocyst is well-documented in the literature. According to the current classification, approximately 30% of all splenic cysts or pseudocysts result from direct abdominal trauma. In addition, chronic pancreatitis leads to change of nearby organs with possible acute and chronic complications including splenic lesions. This unusual complication can occur in both emergent and non-emergent conditions. The useful diagnostic procedures to assess intrasplenic pseudocyst are sonogram, CT scan, splenic scan, and occasionally angiography. However, definite diagnosis of pseudocyst is possible only after splenectomy when the absence of epithelial lining is confirmed histologically. Splenic pseudocyst requires surgical resection. We experienced a 31-year-old man who confirmed of warmness in the left side of back with left upper quadrant abdominal pain for several months. First impression was splenic lymphangioma based on CT scan and sonogram finding. Splenectomy was performed. Microscopic examination revealed splenic pseudocyst with fibrous capsule without epithelial lining.     

Asian variant of CD5+ intravascular large B-cell lymphoma with splenic infarction

A 57-year-old man was admitted with fever and epigastralgia, and presented with splenomegaly and pancytopenia. A CT scan revealed splenic infarctions. There were no lymphadenopathies, skin lesions, or neurological abnormalities. A splenectomy was performed. Bone marrow involvement with hemophagocytosis was noted. The diagnosis of Asian variant of intravascular diffuse large B-cell lymphoma was based on intravascular and sinusoidal distribution of large CD5+ B cells. The patient died of the disease 11 months after onset. To our knowledge, this is the first report of AIVL that presented with splenic infarction. This distinct lymphoma should be included in the differential diagnosis of splenic infarction.     

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.     

Abscesses of the spleen： Report of three cases

Abscess of the spleen is a rare discovery, with about 600 cases in the international literature so far. Although it may have various causes, it is most usually associated with trauma and infections of the spleen. The latter are more common in the presence of a different primary site of infection, especially endocarditis or in cases of ischemic infarcts that are secondarily infected. Moreover, immunosuppression is a major risk factor. Clinical examination usually reveals a combination of fever, left-upper-quadrant abdominal pain and vomiting. Laboratory findings are not constant. Imaging is a necessary tool for establishing the diagnosis, with a choice between ultrasound and computed tomography. Treatment includes conservative measures, and surgical intervention. In children and in cases of solitary abscesses with a thick wall, percutaneous catheter drainage may be attempted. Otherwise, splenectomy is the preferred approach in most centers. Here, we present three cases of splenic abscess. In all three, splenectomy was performed, followed by rapid clinical improvement. These cases emphasize that current understanding of spleen abscess etiology is still limited, and a study for additional risk factors may be necessary.