Is there a correlation between degree of splenomegaly,symptoms and hypersplenism? A study of 218 patients with Gaucher disease

Despite the prevalence of splenomegaly as a sign in many disorders, there have been no studies that correlate the degree of organomegaly with the symptoms generally ascribed to splenic enlargement. The degree of splenomegaly was compared with five overt symptoms of mechanical displacement, i.e. chronic abdominal pain, abdominal discomfort, early satiety, pain while lying on the side, or attacks of acute (colicky) left upper quadrant pains. We have also employed splenomegaly as seen in Gaucher disease as a paradigm to determine whether there is a correlation between the degree of splenomegaly and the parameters of hypersplenism. Although there was a statistically significant correlation between degree of splenomegaly and blood counts, this proved to be clinically negligible. Surprisingly, there was also no correlation between degree of splenomegaly and any of symptoms investigated.     

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.     

A case of splenic pseudocyst complicated by acute pancreatitis

Splenic pseudocyst is a rare disease associated with chronic and acute pancreatitis splenic pseudocyst is treated by distal pancreatectomy and splenectomy. A 47-year old woman with a 10-year history of alcohol abuse presented with epigastric and left upper quadrant pain of 3 days duration. Abdominal CT showed a 4.0×4.5 cm sized cystic lesion in the tail of the pancreas. Analgesics was administrated for the relief of abdominal pain. On the 4th hospital day, the patient complained more of left upper quadrant pain, so we took follow up CT scans. On follow up CT, one large splenic pseudocyst with size of 9.5×4.5×10.0 cm was noted. The patient was treated conservatively by percutaneous catheter drainage and discharged on the 13th hospital day. This case is the first case report of splenic pseudocyst treated conservatively, not by surgery in Korea.     

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.     

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.     

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.     

Case report: splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease.

While acute splenic sequestration and splenic infarction are commonly observed in infants and young children with sickle cell anemia, they are rarely experienced by adult hemoglobin S homozygotes because the recurrent splenic infarction that takes place during childhood is typically followed by scarring, atrophy, and splenic fibrosis. Both acute splenic sequestration and splenic infarction do remain relatively common in adults with the other sickle hemoglobinopathies. These episodes are almost certainly a consequence of the persistently enlarged and distensible spleens that often remain present in these conditions. In this report, the authors describe two adult patients with hemoglobin SC disease: one who developed acute splenic sequestration and one with splenic infarction. In neither case was there a history of recent air travel or exposure to altitude. The clinical course of these two syndromes is presented, and the hematologic, radiologic, and pathologic manifestations are discussed. Because they can sometimes be difficult to distinguish from one another, and because a failure to identify acute splenic sequestration can be catastrophic, these two entities must be included in the differential diagnosis for any hemoglobin SC patient who present with an unexplained fall in hemoglobin, left upper quadrant pain, unexplained fever, or symptomatic splenomegaly.     

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.     

Volvulus d’une rate voyageuse chez l’adulte : à propos d’un cas au CHU de Yopougon (Abidjan-Côte-d’Ivoire)

Wandering spleen is an acquired or congenital anomaly characterized by a defect of the anatomical strictness of the spleen with neighboring organs owing to its long pedicle, the spleen is located on the outside of the upper left quadrant of the abdomen. Sometimes asymptomatic, the spleen in ectopic position may manifest itself by an abdominal or pelvic mass, chronic or acute abdominal pain, in relation to infarction from the torsion of splenic pedicle. Preoperative diagnosis of wandering spleen is easy, thanks to the contribution of non-invasive imaging procedures. We report a case of a wandering spleen complicated by the torsion of splenic pedicle in a young woman who was treated by splenectomy.     

Acute splenic sequestration crisis in adults with hemoglobin S-C disease: a report of nine cases

Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication and one of the leading causes of death in children with sickle cell disease. It is rarely reported in adults with hemoglobin S-C disease and its natural history in these patients has not been well studied. We describe here the clinicopathological features of ASSC in nine adults with hemoglobin S-C disease treated between 1972 and 2000 and followed for a mean period of 9 years (range 0–21 years). ASSC was characterized by acute left upper quadrant abdominal pain, splenomegaly, fever, and a rapid decrease in hematocrit with active erythropoiesis. The hemoglobin decreased by a mean of 4.8 g/dl from the steady state value (range 3.0 to 6.7 g/dl) during ASSC. Two patients failed to respond to transfusion of packed erythrocytes and required urgent splenectomy. There was one fatality—a 76-year-old woman, who died 36 h after admission. There was no recurrence of ASSC in five patients followed for 2, 3, 16, 18, and 21 years, respectively. ASSC in adults is a serious and occasionally, fatal complication of hemoglobin S-C disease. Patients with hemoglobin S-C disease may remain at risk of ASSC into their eighth decade.     

Splenomegaly with sea-blue histiocytosis, dyslipidemia, and nephropathy in a patient with lecithin-cholesterol acyltransferase deficiency: a clinicopathologic correlation

A 31-year-old man with no significant medical history presented with a 5-day history of progressive left upper quadrant abdominal pain. Physical examination revealed a tender guarded abdomen, no icterus, and bilateral corneal “arcus senilis”-like changes. Laboratory workup showed a mild normocytic, normochromic anemia; and target cells were seen in the peripheral blood smear. Serum was turbid; and the lipid profile showed elevated total cholesterol, low high-density lipoprotein cholesterol, and elevated triglycerides. Urinalysis revealed nephrotic range proteinuria with microhematuria. An abdominal computed tomographic scan demonstrated a homogeneously enlarged spleen. The patient was discharged after symptomatic treatment to be followed as an ambulatory patient. Several days later, he returned with severe left upper quadrant pain and was admitted to the surgical service for further evaluation. A splenectomy was performed for a suspected splenic lymphoma. Upon gross examination, spleen was moderately enlarged, weighing 780 g. Sectioning revealed a beefy red cut surface without gross lesions. Wright-Giemsa-stained touch imprints showed many sea-blue histiocytes. A renal biopsy was also performed, demonstrating focal segmental glomerular sclerosis and mesangial expansion with extramembranous and intramembranous deposition of lipids. In the absence of hematologic malignancy and in light of the abnormal lipid profile, a disorder of lipid metabolism was suspected. Histologic and ultrastructural findings in the kidney and spleen raised the likelihood of lecithin-cholesterol acyltransferase (LCAT) deficiency, which was confirmed by the markedly decreased serum LCAT activity and serum LCAT mass. We describe a case with the triad of splenomegaly with sea-blue histiocytes, nephropathy, and dyslipidemia in a patient with LCAT deficiency.     

Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration

The association of sickle cell trait (SCT) and hereditary spherocytosis (HS) has been reported in only 18 patients. Three of these 18 patients experienced splenic infarct or acute splenic sequestration. We report here a 46-year-old African-American male, the oldest reported case to date, who experienced episodes of hemolysis and severe left upper quadrant pain for the past 26 years. The patient had compensated hemolysis with splenomegaly. A CT scan of the abdomen revealed a large infarct in the spleen. The diagnosis of SCT was confirmed with isoelectric focusing, cation exchange and reverse-phase HPLC. The presence of a silent, interacting globin variant as the cause of hemolysis and sickling in the spleen was ruled out by sequencing of the alpha1-, alpha2- and beta-globin genes. The diagnosis of HS was established by an osmotic fragility test. The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case.     

Peritonitis secondary to ruptured splenic abscess: A grave complication of typhoid fever

Splenic abscesses are increasingly being identified, possibly due to widespread use of imaging modalities in clinical practice. The commonest clinical features are high grade fever and exclusively localised left upper quadrant abdominal pain. These symptoms are similar to most infectious diseases prevalent in the tropics, making imaging by ultrasonography or computer tomography a necessity in the diagnosis. There are reports from different geographic areas on splenic abscesses associated with typhoid fever. We reported ruptured splenic abscess presenting with peritonitis as a rare and grave complication of typhoid fever.     

Localized giant pseudopolyposis of the colon in ulcerative colitis

Recently a patient with ulcerative colitis developed abdominal pain and a left upper quadrant mass. A⁶⁷Ga-citrate scintiscan showed increased activity over the mass. A barium enema demonstrated retrograde obstruction at the splenic flexure and intraluminal multilobulated tissue masses. The total abdominal colectomy specimen showed localized giant pseudopolyposis at the splenic flexure. This condition is a rare local complication of both ulcerative and granulomatous colitis. It resembles a villous adenoma on barium enema and, although inflammatory, may simulate a colonic carcinoma. When symptomatic, local resection may be sufficient treatment.     

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.     

A case of acute pancreatitis presenting with electrocardiographic signs of acute myocardial infarction

A 71-year-old man presented with left upper quadrant abdominal pain. Serial electrocardiograms (ECGs) demonstrated an evolving left bundle branch block, a sign of acute myocardial infarction (AMI). However, a coronary angiogram demonstrated minimal coronary artery disease, and serum troponin T was undetectable in serial serum measurements. Later, serum pancreatic enzyme levels were elevated and a computed tomography scan of the abdomen was consistent with pancreatitis. In patients presenting with acute pancreatitis and ECG changes suggesting AMI, measurement of serum troponin T concentrations can aid in differentiating ECG changes driven by acute pancreatitis from those of true myocardial ischemia or infarction.     

Le syndrome de la rate baladeuse : cas clinique

IntroductionWandering spleen is a rare condition that occurs when there is an acquired or congenital hypelaxity of the suspensory ligaments of the spleen, resulting in its migration to any abdominal or pelvic position.Case reportA 43-year-old woman presented with symptoms of acute abdominal pain. At physical examination, a splenomegaly was found in the left hypochondria, thereafter followed by a secondary migration of the lump to a periumbilical location. Computed tomography revealed a wandering spleen with vascular pedicle torsion. Splenopexia was perfomed.ConclusionA wandering spleen is most frequently observed in children and young women. This rare diagnosis should be considered facing a recurrent abdominal pain syndrome, a splenomegaly accompanied with pain, or a pelvic lump. A wandering spleen may lead to torsion of the vascular pedicle of the spleen, a chronic volvulus with portal hypertension, or even a splenic infarction. The diagnosis is performed by computed tomography. Wandering spleen is treated surgically, by splenopexy or splenectomy.     

The 'true' splenic wanderer.

Wandering spleen is an unusual entity and remains an elusive clinical diagnosis. Among the modern imaging modalities including computed tomography, magnetic resonance imaging, nuclear scans and ultrasonography, the latter appears to be the least invasive and the most effective in reaching a definitive diagnosis. A patient with 'true' wandering spleen who presented with chronic, intermittent abdominal pain, weight loss and a right lower quadrant mass that was interpreted as a pelvic lymphoma or a primary pelvic malignancy on computed abdominal tomography (CAT) scan is presented. Abdominal ultrasonography conducted a few weeks before the CAT scan showed a normal splenic shadow in the left upper abdomen.     

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.     

An exceptional cause of left lower quadrant abdominal pain

Acute appendidtis is an exceptional cause of left lower quadrant abdominal pain. Computed tomography scan is the key to its diagnosis and helps to establish its early treatment. We present a case of a 35-year-old male patient who presented acute appendicitis with redundant and loosely attached cecum which was diagnosed based on his left lower quadrant abdominal pain.