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Splenomegaly at a United States County Hospital: diagnostic evaluation of 170 patients.

Splenomegaly was studied at a municipal teaching hospital in an 11-year retrospective review. The 170 patients were classified into six diagnostic groups. The associated clinical and laboratory features were tested for statistical association (X2), to determine predictive values. Hepatic diseases caused 36% of the splenomegaly; hematologic, 35%; infectious diseases (ID), 16%; inflammatory, 5%; primary splenic, 4%; and other, 3%. The acquired immunodeficiency syndrome (AIDS) occurred in 54% of patients with ID. Hematologic diseases were significantly associated (P < 0.01) with massive splenomegaly, left upper quadrant (LUQ) abdominal tenderness, and all blood "cytoses." The most common disease with massive splenomegaly was myelofibrosis. Surprisingly, hepatic diseases caused 29% of massive splenomegaly. Hepatic diseases were significantly associated (P < 0.01) with hepatomegaly, abnormal liver-function tests (LFT), and blood "cytopenias." Compared with previous reports, both congestive heart failure and endocarditis now rarely cause splenomegaly. All blood "cytopenias" had highly significant associations (P < 0.01) only with hepatic diseases, which suggests that hypersplenism remains a useful concept for the splenomegaly of liver disease.

Adolescent↗

Splenomegaly at a university hospital compared to a nearby county hospital in 317 patients.

Splenomegaly and massive splenomegaly were diagnostically evaluated retrospectively at Stanford University Hospital in 147 patients over 8 years and compared to the nearby county hospital (Santa Clara Valley Medical Center; VMC) in 170 inpatients over 11 years. Hematologic diseases at Stanford (data for VMC in parentheses) occurred in 66% (35%; p < 0.001) of the patients with splenomegaly and in 84% (54%; p < 0.001) of those with massive splenomegaly. Hepatic diseases occurred in 9% (36%; p < 0.001) of the patients with splenomegaly and in 5% (29%; p < 0.001) of those with massive splenomegaly. Splenectomy was performed in 71% of the patients at Stanford and in 9% of those at VMC (p < 0.001). The combined Stanford-VMC series showed significant associations (p < 0.01): for hematologic diseases with massive splenomegaly, lymphadenopathy, and blood cytoses; for hepatic diseases with hepatomegaly, cytopenias as hypersplenism, and abnormal liver function tests, and for infectious diseases with fever.

Adolescent↗

Duplex sonography in splenomegaly.

The aetiology of splenomegaly is seldom clear from either clinical or imaging assessment of the spleen. In the majority of cases sonographic assessment of the spleen produces a homogeneous enlargement of variable echodensity, but with very poor correlation with pathology. A study has been undertaken to assess the Doppler characteristics of the splenic artery in splenomegaly, excluding cases of portal hypertension in an attempt to provide further diagnostic information. Duplex assessment of 18 normal subjects and 10 patients with splenomegaly due to five different causes was undertaken. Analysis of waveform, peak frequency and pulsatility index were compared and failed to demonstrate any significant change. In the normal subject there is a broad systolic spectrum related to the tortuosity of the splenic artery, with persistence of forward flow throughout diastole, a reflection of the low peripheral resistance of the spleen. In splenomegaly the broad systolic spectrum is unchanged, since vessel tortuosity persists. Pathological and physiological assessment of the spleen in splenomegaly shows that a low resistance circulation persists, explaining maintenance of the diastolic flow pattern in splenomegaly. Increased blood flow to the spleen in splenomegaly occurs either as a result of an increase in vessel diameter or an increase in flow velocity, or a variable combination of the two which does not seem to be governed by specific pathology. An increase in peak frequency in some cases reflected some increase in flow velocity but provided no useful correlation. Analysis of the pulsatility index supported the above findings without adding further information.

Adolescent↗

Sarcoidosis and giant splenomegaly.

Splenomegaly is often a manifestation of sarcoidosis, but giant splenomegaly is rare. Only 25 cases of sarcoidosis presenting as massive splenomegaly have been reported. Splenectomy was performed before the diagnosis of sarcoidosis was made in more than half of these patients. Serious complications of giant splenomegaly including rupture, severe thrombocytopenia and hemolytic anemia were not consistently present preoperatively. A case of disseminated sarcoidosis presenting with giant splenomegaly and normal chest roentgenograms is described. Prednisone therapy improved the patient's pulmonary function testing while constitutional symptoms, splenomegaly and elevated angiotensin-converting enzyme levels completely resolved. Sarcoidosis should be considered in the differential diagnosis of massive splenomegaly. A trial of steroids is warranted as initial treatment, and in selected cases may prevent unnecessary splenectomy, thereby precluding a compromise in host defenses against encapsulated bacteria.

Adult↗

Surgical management of massive splenomegaly.

Recent reports document an increased mortality and morbidity associated with splenectomy for massive splenomegaly (<1000 g), with a morbidity that is 2 to 10-fold higher than that seen for splenectomy for normal size spleens. Preoperative angiographic embolization of the splenic artery has been advocated as a means to decrease this morbidity and mortality. In a retrospective review of 100 splenectomies performed at Kaiser Permanente (Los Angeles, CA), 20 were performed for splenomegaly, average weight 1811 g (1050-3700 g), and 80 were normal sized spleens. Mortality for normal sized spleens is 1.25 per cent, and for those performed for splenomegaly is zero. Likewise, the morbidity for splenectomy of normal sized spleens was 21.25 per cent, but for splenomegaly, a 20 per cent morbidity rate was observed. Average blood loss with splenomegaly was 696 mL, slightly higher than the 600 mL blood loss for normal sized spleens. Sixty-seven per cent of patients with splenomegaly required no transfusion, and none required more than two units. Of patients with normal size spleens, 75 per cent required no transfusion, and 96 per cent required two units or less. Splenectomy for splenomegaly is possible without an increase in morbidity or mortality. In this series, preoperative embolization was not performed; however, the morbidity and mortality rates compare favorably with series in which it was performed. Preoperative embolization of the splenic artery may be unnecessary and may expose the patient to additional expense, risk, and discomfort.

Adolescent↗

[Splenomegaly in sarcoidosis: clinical features and outcome. Analysis of 17 cases].

PURPOSE: To describe the clinical features, biological datas and outcome of patients with systemic sarcoidosis and splenomegaly. METHODS: A retrospective analysis of 17 patients presenting splenomegaly and sarcoidosis with histological proof. RESULTS: Splenomegaly was clinically perceptible in 13 patients, with a spleen size that extended 4 cm or more below the costal margin in 11 patients. It was painful in five cases. The more frequent clinical features are constitutional symptom (fever in 9 cases) and hepatomegaly (N =7). Chest X-ray showed bilateral hilar lymphadenopathy in nine patients and no abnormality in five cases. Serum angiotensin converting enzyme levels were elevated in 81% of cases. Thrombopenia (N =5) and hypersplenism (N =5) were also observed. Corticosteroid were given to 88% with a good clinical and biological response including a decrease in the spleen volume. Corticotherapy and splenectomy (performed in two patients to rule out lymphoma) didn't change outcome of disease. Sarcoidosis is often chronical (82%) and extensive. CONCLUSION: Splenomegaly may be present in sarcoidosis. Management is not standardized. Corticosteroid is indicated for symptomatic or massive splenomegaly. Splenomegaly is frequently in chronic and extensive sarcoidosis.

Adrenal Cortex Hormones↗

Hyperreactive malarial splenomegaly in expatriates.

Hyperreactive malarial splenomegaly, one of the major causes of splenomegaly in tropics, has been often reported in expatriates of non-tropical settings. Essential features are recurrent malarial infection, overproduction of IgM and hyperplasia of lymphoreticular system. The practice of diagnosing the condition by exclusion of obvious causes of splenomegaly in the tropics has been abandoned. There are specific criteria for the diagnosis. Huge splenomegaly >10 cm below costal margin, serum IgM more than 2 x standard deviation (2SD) above the local mean, high titre of malarial antibodies and response to antimalarial drugs are the cornerstones of the diagnosis. Splenic lymphoma with villous lymphocytes coexists with this condition and it should always be considered in the differential diagnosis of unresponsive or poorly responsive cases of hyperreactive malarial splenomegaly. Condition with fever and acute haemolysis in HMS has been termed as Fulminant tropical splenomegaly syndrome. Treatment of the condition depends on antimalarial (chloroquine/ proguanil/ pyrimethamine) chemoprophylaxis for 1 year or more.

Animals↗

Laparoscopic splenectomy for massive splenomegaly.

BACKGROUND: Laparoscopic splenectomy (LS) is the preferred operative approach for diseases involving normal-sized spleens. Our experience with laparoscopic splenectomy in the setting of massive splenomegaly is presented. METHODS: A prospective review of patients undergoing LS for massive splenomegaly was conducted. Massive splenomegaly (MS) in adults was defined as a craniocaudal length >or=17 cm or a morcellated weight >or=600 g. In children, spleens measuring fourfold larger than normal for age were considered massive. RESULTS: Forty-nine patients with MS were treated with LS. The most common primary diagnoses were lymphoma and leukemia. Mean splenic length was 20 cm (15 to 27 cm), with weights ranging from 600 to 4,750 g. Twelve patients with supermassive splenomegaly (length >22 cm) required a hand-assisted laparoscopic approach. There were no conversions to open surgery. Mean operating time was 171 minutes (90 to 369). Mean blood loss was 114 cc (<30 to 600 cc). Average length of stay was 2.3 days (1 to 16). Minor postoperative complications occurred in 3 patients. CONCLUSIONS: Laparoscopic splenectomy in the setting of splenomegaly is safe and appears to minimize perioperative morbidity. In patients with supermassive splenomegaly, a hand-assisted laparoscopic approach may be required.

Adult↗

Is splenectomy for massive splenomegaly safe in children?

PURPOSE: To study and analyze the causes, etiology, morbidity, mortality and therapeutic value of splenectomy performed for massive splenomegaly in children. METHODS: The medical records of 115 children less than 18 years old who had splenectomy for various hematological disorders were reviewed. Twenty of them had splenectomy for massive splenomegaly (spleen weight > or =1,000 g). The records of these were reviewed for age at operation, gender, hematological diagnosis, indication for splenectomy, operative procedures, postoperative complications, and outcome. RESULTS: Twenty children had splenectomy for massive splenomegaly. There were 16 males and 4 females. Their ages ranged from 4 to 15 years (mean 11.2). Twelve had sickle cell disease, 5 had sickle-beta-thalassemia, 1 had beta-thalassemia major, 1 had thalassemia intermediate, and 1 had chronic myeloid leukemia. The indications for splenectomy were hypersplenism in 11, recurrent splenic sequestration crisis in 8, and splenic abscess in 1. The transfusion requirements in the patient with beta-thalassemia major decreased markedly postoperatively from 18 transfusions/year to only 4 transfusions/year; and for those with hypersplenism, there was a marked improvement in their blood parameters following splenectomy. The patient with thalassemia intermediate required no more blood transfusions. There was no mortality. The immediate postoperative morbidity was 10% for those with massive splenomegaly compared with 6.3% for those with splenomegaly <1,000 g. CONCLUSIONS: With good perioperative management, splenectomy in children with massive splenomegaly is both safe and effective.

Adolescent↗

Mediastinal lymph node enlargement and splenomegaly in primary hypogammaglobulinaemia.

The computed tomography (CT) scans of 37 patients with primary hypogammaglobulinaemia were reviewed to determine the frequency of enlarged mediastinal lymph nodes and splenomegaly in this group. None of the 10 X-linked Agammaglobulinaemia (XLA) patients had enlarged nodes and only one had splenomegaly. Eleven of the 27 Common Variable Immunodeficiency (CVID) group had enlarged nodes (41%) and 13 had splenomegaly (48%). There was no significant correlation between the presence of enlarged nodes and splenic enlargement. Twenty-two patients had bronchiectasis but the presence of bronchiectasis did not correlate with the presence of either splenomegaly or lymphadenopathy. Three to 6 years follow-up is available for 36 of the 37 patients and none of this group have developed lymphoma or other malignancy. Enlarged mediastinal nodes and/or splenomegaly are frequently found in patients with CVID and are usually due to a benign, non-neoplastic, process. Mediastinal lymph node enlargement is not a feature of XLA and splenomegaly is unusual in this condition.

Adolescent↗

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.

Abdominal Pain↗

Splenomegaly in murine trypanosomiasis: T cell-dependent phenomenon.

Splenomegaly resulting from Trypanosoma musculi infection was found to be dependent upon a functioning T-lymphocyte system. When both humoral and cell-mediated immune systems were suppressed by treatment with cyclophosphamide 2 days after infection, splenomegaly was inhibited for about 6 days. However, when only humoral immunity was suppressed by treatment with cyclophosphamide 3 days before infection, splenomegaly still occurred. In addition, splenomegaly was absent in congenitally athymic nude mice. Nude mice and immunologically intact heterozygote mice were also sacrificed at varying times after infection, and spleens were examined histologically. A lymphocytic hyperplasia was observed in immunologically intact mice but not in nude mice. These data indicate that splenomegaly of T. musculi infections is T cell dependent and that splenomegaly is the result of a proliferation of B and/or T lymphocytes.

Animals↗

[Splenomegaly and plasma lipids].

UNLABELLED: Lipid metabolism is influenced by several factors, among them the spleen is supposed to play some spleen. It has been noticed that in the asplenia the level of the triglycerides is raised. On the other hand the patients with myeloproliferative process and splenomegaly have lower level of serum lipids compared with the general population. The aim of this clinical study was to estimate the level of: total cholesterol (TC), HDL-cholesterol (HDL-C), LDL-cholesterol (LDL-C), and triglycerides (TG), in group I which consisted of 17 patients with splenomegaly, which had developed during portal hypertension caused by liver cirrhosis, and group II 7 patients with splenomegaly caused by myeloproliferative disease. These patients were compared with the control group III of 25 patients without evidences of metabolism disturbances or splenomegaly. There were no statistically significant differences of the mean values of: TC, HDL-C, LDL-C, and TG between groups I and II. The levels of all serum lipids were lower in the patients with splenomegaly (groups I, II) irrespective of causal factor, compared with the control group III. CONCLUSION: The changes in the serum lipids in the patients with splenomegaly indicate the potential role which the spleen can play the spleen in the lipid metabolism.

Adult↗

Frequency and etiology of splenomegaly in adults seeking medical advice in Combined Military Hospital Attock.

BACKGROUND: The concept that a palpable spleen can be associated with disease is natural, because this organ normally is not palpable, but may become so under certain circumstances. Questions concerning the frequency, etiology and significance of finding a palpable spleen are raised from time to time. These factors vary with geographical distribution of tropical diseases, and other diseases prevalent in the area. We conducted this study in the department of medicine, at Combined Military Hospital Attock from Sept 2002 to Sept 2003, to find out the frequency and etiology of splenomegaly in our setup. METHODS: All patients above the age of 13 years seeking advice of medical specialist in our hospital were included. History and clinical examination was performed and relevant investigations were carried out to establish the diagnosis. RESULTS: 1400 patients were examined. Splenomegaly was present in 96 cases (6.8%). Male to female ratio was 4:1. 83% patients had a palpable spleen of 1-5 cm while 8.5% had >10 cm. Different types of anemia constituted 29% of cases, malaria 25%, bacterial infections 18.6% and hematological malignancies 16%. Hepatitis/cirrhosis related splenomegaly was seen in 9.4% cases while 1% had tropical splenomegaly. CONCLUSION: Different types of anemias, malaria, bacterial infections and hematological malignancies are common causes of splenomegaly in our setup. Splenomegaly should be investigated further since all the common causes are treatable.

Adolescent↗

Haematological parameters in sickle cell anaemia patients with and without splenomegaly.

BACKGROUND: The aim is to investigate the clinical and haematologic effects of persistent splenomegaly (PS) and splenectomy in a population of Nigerians with sickle cell anaemia (SCA). MATERIALS AND METHODS: SCA patients attending the Haematology clinic of the OAUTHC, Ile-Ife, were examined physically and haematologically. Patients with PS were compared with age and sex-matched controls without splenomegaly. Foetal haemoglobin (HbF), packed cell volume (PCV), reticulocyte count and the frequency of blood transfusion were noted for both groups. The clinical effects of splenectomy were documented in three patients who underwent the procedure because of symptomatic massive splenomegaly (> or = 10 cm). RESULTS: Seventy-one patients, 40 males and 31 females, aged 16-48 (median, 21) years were studied. Nineteen (26.8%) had PS ranging from 3-22 cm (mean +/- SD = 9.4 +/- 4.4 cm; median = 7 cm). Ten of these had massive splenomegaly (spleen > or = 10 cm) varying from 10-22 cm (mean +/- SD = 12.3 +/- 3.8 cm; median = 12 cm). No significant differences were found between the mean values of HbF, PCV, reticulocyte count, frequencies of pain crisis, transfusion requirement, weights and heights in SCA with PS and age-sex matched controls without PS. Annual transfusion requirement showed a mild negative correlation with splenic size (r = -0.06), which was not statistically significant (p = 0.882). Significant haematologic improvement was confirmed in the 3 patients who underwent elective splenectomy for splenomegaly-induced hypersplenism. CONCLUSIONS: We conclude that massive persistent splenomegaly is a recognised cause of significant morbidity in SCA patients as evident in the patients that underwent splenectomy. However, where it is mild to moderate, PS may not have significant adverse effect on the overall clinical picture of the disorder.

Adolescent↗

[Difficulties in the differential diagnosis of splenomegaly].

The authors discuss diagnostic difficulties that may occur in the interpretation of splenomegaly. In some cases, other tumorous formations, palpable in the left hypochondrium (renal tumor and retroperitoneal fibroma in cases demonstrated) may be taken for splenomegaly. In other cases, diagnostic difficulties may be related to establishing the cause of genuine splenomegaly. Out of 10 patients with genuine splenomegaly, the cause of which could not be ascertained at the prehospital stage, the enlargement of the spleen in 2 was due to liver cirrhosis, in 3, to subleukemic myelosis, in 1, to splenic tuberculosis, in 2, to multiple capillary angiomas of the spleen, in 1, to chronic monocytic leukemia, and in 1 patient, to splenic echinococcosis. In 3 patients out of the 10, the diagnosis was verified by surgery followed by histological examination of the spleen. The conclusion is made about the necessity of the use of clinical and laboratory examinations, of patients with obscure "splenomegaly" together with the use of x-ray and ultrasonography of the spleen, kidneys, gastrointestinal tract, puncture of the bone marrow and spleen. In some cases, splenectomy may be performed along with histological examination of the spleen as the final stage of differential diagnosis of splenomegalies.

Adult↗

Induction of splenomegaly in mice by killed Coxiella burnetii cells.

Splenomegaly induced in mice inoculated intraperitoneally (i.p.) with purified formalin-killed phase I and phase II Coxiella burnetii (C.b.) cells was dose-dependent. The phase I cells induced higher splenomegaly than phase II cells. The splenomegaly-inducing ability of phase I cells was reduced upon incubation with phase I but not with phase II antiserum, whereas the phase II cells preincubated with phase I or phase II immune sera induced higher splenomegaly than the phase II cells alone. Phase I cells caused lower splenomegaly in mice previously immunized with C.b. The splenomegaly-inducing ability of phase I cells was abolished by mild acid hydrolysis, by treatment either with phenol-chloroform-petroleum ether (PCP) or with a chloroform-methanol (CM) mixture. However, either the CM or the PCP-treated phase I cells retained their capacity to protect mice challenged with virulent phase I C.b.

Alkanes↗

Serological and immunological investigations in patients with gross splenomegaly from the Gabon.

Serological investigations (immunoglobulin, haptoglobin, cryoglobulin, and antibody determination against Plasmodium falciparum and P. malariae antigens) were performed in 64 adults of the Albert-Schweitzer-Hospital, Lambarene, Gabon. The patients were referred consecutively for ultrasound examination of the upper abdominal tract. 31 patients had clinically and sonographically an enlarged spleen, whereas 34 had a normal-sized spleen. 18 patients were regarded as having a gross splenomegaly without an obvious underlying cause (tropical splenomegaly (TSS]. No significant differences were seen between the patient groups with regard to immunoglobulin M or antiplasmodial antibody concentration. Thus, a causal association of splenomegaly with chronic malaria infections could not be established. The haptoglobin levels were significantly (P less than 0.01) reduced in patients with splenomegaly. Peripheral T-lymphocyte subsets as defined by monoclonal antibodies showed in all 4 cases with gross splenomegaly examined distinct abnormalities. Tropical splenomegaly is thought to encompass a variety of diseases, most of them presenting an intermediate stage of reactive to autonome disorders of the lymphatic system.

Adolescent↗