Spontaneous rupture of the spleen in AL amyloidosis.

The frequency of splenic involvement in AL amyloidosis is not precisely known. However, splenomegaly has been reported in 4-13% of patients. We report four cases of spontaneous splenic rupture in patients with AL amyloidosis. Splenic rupture was the initial manifestation of the disease in one of these patients. The other three experienced splenic rupture during or after high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM/SCT): one during stem cell mobilization with G-CSF prior to HDM/SCT and two after hematopoietic recovery following treatment. Two of the four patients had Factor X deficiency, the most common coagulation abnormality associated with AL amyloidosis. All four patients underwent splenectomy without significant postoperative complications. Splenic rupture in AL amyloidosis as a complication of aggressive treatment with HDM/SCT has not been reported previously.

Risks and benefits of splenectomy in myelofibrosis with myeloid metaplasia: a retrospective analysis of 26 cases.

BACKGROUND AND OBJECTIVES: To evaluate the outcomes of splenectomy in myelofibrosis and myeloid metaplasia (MMM). METHODS: We retrospectively reviewed our records of 26 patients with MMM who underwent an open splenectomy at Boston University Medical Center between 1979 and 1995. Fourteen patients had agnogenic myeloid metaplasia (AMM) and 12 had myelofibrosis with antecedent myeloproliferative disorders (MF). The main indications for splenectomy were progressive transfusion-dependent anemia, painful splenomegaly, and hypercatabolic symptoms associated with cytopenia. RESULTS: Median time to splenectomy after the diagnosis of MMM was 29 months ranging from 1 to 96 months. Three patients (11%) died within 1 month after the surgery because of sepsis. The most common peri- and postoperative complications were pneumonia and other bacterial infections (42%), cardiac events (19%), acute bleeding (15%), ileus (15%), and venous thrombosis (12%). Of the eight surviving patients who underwent splenectomy for transfusion dependent anemia, six (75%) had improvement in their hematocrit levels with abolishment of blood transfusions. A durable symptomatic palliation was achieved in all patients. Liver enlargement was noted in seven patients at 1-year evaluation. None of these patients developed hepatic failure. Leukemic transformation occurred in 8 of 18 patients (44%) postsplenectomy. The median overall survival for the entire group was 58.5 and 28 months from the diagnosis of MMM and the time of splenectomy, respectively. There was no difference in survival rates between patients with AMM and MF. CONCLUSIONS: Splenectomy is an effective palliative procedure with an acceptable morbidity in selected patients with MMM. Progressive transfusion-dependent anemia should also be considered an indication for splenectomy in the absence of leukemic evolution.

Comparative response to splenectomy in Coombs-positive autoimmune hemolytic anemia with or without associated disease.

We reviewed our experience in 30 patients with direct Coombs-positive (DAT+) autoimmune hemolytic anemia (AHA) who underwent splenectomy. Twelve patients had idiopathic "warm" AHA (group I) and 18 had AHA associated with systemic diseases (group II). Complete response to splenectomy was defined as having normal hemoglobin and reticulocyte count lasting for at least 6 months without subsequent medical therapy. Subnormal but greater than 50% improvement in these parameters with or without medical therapy was considered to be a partial response. Median age was 64 (23-81) in group I and 68 (23-76) in group II. Median follow-up duration was 18 and 10.9 months, respectively. Nine of 11 (82%) evaluable patients with idiopathic AHA and 3 of 16 (19%) patients with associated disease achieved a complete response. Partial response was obtained in 2 (18%) and 6 (37%) patients in groups I and II, respectively. Both complete-response and overall-response rates were statistically different between two groups (P = 0.001 and 0.02). Postoperative courses of group I patients were uneventful except for one who developed a subphrenic abscess. Five patients in group II developed bacterial infections, which were mostly pneumonias. Our findings indicate that splenectomy is an effective treatment approach with low morbidity and mortality in patients with refractory idiopathic AHA. It should, however, be considered cautiously in AHA patients with underlying systemic diseases because of its decreased efficacy and increased surgical morbidity in this subgroup.

Occlusive colon carcinoma: virtual colonoscopy in the preoperative evaluation of the proximal colon.

PURPOSE: To evaluate the use of preoperative virtual colonoscopy to examine the proximal colon in patients with distal occlusive carcinomas, defined as cancers that cannot be traversed endoscopically. MATERIALS AND METHODS: Twenty-nine patients with occlusive colorectal carcinomas underwent preoperative virtual colonoscopy with use of a standard protocol. Patients with acute bowel obstruction were excluded. Results of virtual colonoscopy were compared with the findings of preoperative colonoscopy, preoperative barium enema examination, intraoperative colon palpation, histopathologic outcome, and postoperative colonoscopy and barium enema examination, where possible. RESULTS: Virtual colonoscopy helped identify all 29 occlusive carcinomas and demonstrated two cancers and 24 polyps in the proximal colon. Both synchronous cancers were confirmed intraoperatively and resected. Postoperative conventional colonoscopy in 12 patients confirmed 16 polyps identified at virtual colonoscopy and demonstrated two subcentimeter polyps missed at virtual colonoscopy. Postoperative barium enema examination was performed in two patients and helped confirm two polyps identified at virtual colonoscopy. Virtual colonoscopy successfully demonstrated the proximal colon in 26 of 29 patients examined compared with preoperative barium enema examination, which failed to adequately demonstrate the proximal colon in any patient examined. CONCLUSION: Virtual colonoscopy is a feasible and useful method for evaluating the entire colon before surgery in patients with occlusive carcinomas.

Is splenectomy more dangerous for massive spleens?

BACKGROUND: Reports vary about whether risks are greater for removal of massive (> or = 1500 g) spleens than for smaller (< 1500 g) spleens. We sought to determine the hazards of splenectomy. METHODS: We reviewed 223 consecutive adults with elective splenectomies for hematologic diseases. Morbidity and mortality rates were combined with published data to create a meta-analysis. RESULTS: Patients with massive spleens are more likely to have postoperative complications (relative risk [RR] 2.1, 95% confidence interval [CI] 1.3 to 3.4; P = 0.003) and death (RR 4.7, 95% CI, 1.5 to 15.1; P = 0.01). However, when the investigation is restricted to comparable diagnoses, patients with massive spleens do not differ from those with smaller spleens regarding complications (RR 1.4, 95% CI, 0.8 to 2.7; P = 0.3) or mortality (RR 2.1, 95% CI, 0.5 to 9.7; P = 0.4). These observations are confirmed by metaanalysis. Furthermore, multivariate analysis indicts age as a critical risk of complications and death. CONCLUSIONS: Increased age and underlying illness are the predominant factors associated with morbidity and mortality following splenectomy for hematologic disease. Adjusting for age and diagnosis, spleen size is not a hazard.

Loss of expression of a 55 kDa nuclear protein (nmt55) in estrogen receptor-negative human breast cancer.

We have identified and characterized a 55 kDa nuclear protein (referred to as nmt55) from human breast tumors and MCF-7, human adenocarcinoma breast cell line, using site-directed monoclonal antibodies. Measurements of estrogen receptors (ER) and progesterone receptors (PR), by ligand binding assays, in cytosols of 63 human breast tumors permitted classifications of these tumors into four phenotypes (ER+/PR+, ER+/ PR-, ER-/PR-, ER-/PR+). Nuclear protein (nmt55) expression in these tumors, as determined from Western blot analyses, showed a statistically significant association (p = 0.001) with tumor hormonal phenotype. Review of the pathologic characteristics of tumors analyzed suggested that lack of nmt55 expression was significantly associated with mean tumor size (p < 0.03), mean ER (p = 0.001) and mean PR (p < 0.002), but was not associated with tumor stage, grade, or type. To further study this protein, we cloned and sequenced a 2.5 kb cDNA using a monoclonal antibody to nmt55. The complete predicted open reading frame encodes a protein with 471 amino acids and a calculated molecular mass of 54,169 Da. The deduced amino acid sequence exhibited unique regions rich in glutamine, histidine, arginine, and glutamic acid. Northern blot analysis of RNA from MCF-7 cells and ER+/PR+ human breast tumors showed a 2.6 kb mRNA. Southern blot analysis suggested the presence of a single copy of this gene. Chromosomal mapping, using fluorescent in situ hybridization (FISH), located nmt55 gene to the X chromosome, region q13. The extensive homology between nmt55 and RNA binding proteins suggested that nmt55 may be involved in hnRNA splicing. The strong association observed between expression of nmt55, tumor hormonal phenotype, mean tumor size, mean ER, and mean PR content suggests that loss of nmt55 expression may be related to events involved in hormone insensitivity, tumor differentiation, and unregulated tumor cell growth and metastases.

Results of a phase I trial of a recombinant vaccinia virus that expresses carcinoembryonic antigen in patients with advanced colorectal cancer.

BACKGROUND: The inadequacy of systemic treatments of advanced colorectal cancer has aroused interest in biologic therapy. Recent animal models have demonstrated the efficacy and safety of a recombinant vaccine that contains vaccinia and the gene for carcinoembryonic antigen (rV-CEA). METHODS: A phase I clinical trial of rV-CEA was conducted to assess vaccine toxicities, the maximum tolerated dosage, resulting immune activities, and tumour responses. A dose-escalation protocol was devised for three concentrations. Six patients per dosage were each to receive three vaccinations. RESULTS: Seventeen patients with advanced colorectal cancer received a total of 44 vaccinations. Mild local and systemic reactions-comparable to those seen with vaccinia alone-were observed and were typically associated with the first vaccination. No significant complications or deaths were caused by the rV-CEA. In particular, no autoimmune colitis developed, nor did leukopenia occur, despite some homology between CEA and leukocyte antigens. All three vaccine concentrations were equally well tolerated. Most patients demonstrated tumor progression by clinical and radiographic parameters and by CEA levels. Immune assays are pending. CONCLUSIONS: This phase I trial demonstrated the safety of rV-CEA in patients with advanced colorectal cancer. Future clinical studies are warranted and will likely be influenced by investigations of the immune responses to the vaccine.

Risks of synchronous gastrointestinal or biliary surgery with splenectomy for hematologic disease.

BACKGROUND: The addition of splenectomy to a gastrointestinal (GI) operation may have an adverse effect on mortality, morbidity, and even survival. OBJECTIVE: To determine the risks of the converse: synchronous GI surgery appended to splenectomy for hematologic diseases. DESIGN: Retrospective cohort. SETTING: Multiple hospitals comprising an affiliated surgical training program. PATIENTS: Consecutive sample of 207 adults (mean age, 49 years) with splenectomies for hematologic diseases. INTERVENTION: Splenectomy and concomitant GI or biliary surgery (group 1, n=19) and splenectomy alone (group 2, n=188). MAIN OUTCOME MEASURES: Length of hospital or intensive care unit stay, later operations, postoperative infections, postoperative abdominal abscess, major complications, and death. RESULTS: Preoperative and intraoperative factors were similar in both groups. Operative mortality was 3 of 19 in group 1 and 8 of 188 in group 2 (p=.07). The mean number of major complications tended to be higher in group 1 (1.5 vs 0.5, P=07). Despite no difference between the incidences of overall postoperative infections, patients in group 1 were much more likely to develop an abdominal abscess (4 of 19 vs 3 of 188, P=.002). Logistic regression established that patients undergoing splenectomy and synchronous GI or biliary surgery were 25 times more likely to develop an intra-abdominal abscess than were patients with splenectomy alone, even controlling for confounding factors (odds ratio, 24.7; 95% confidence interval, 3.1 to 196; P=.002). CONCLUSIONS: Synchronous GI or biliary surgery with splenectomy for hematologic disease increases the risk of intra-abdominal abscess and should be avoided. Complication and mortality rates may also be increased.

A complementary role for thallium-201 scintigraphy with mammography in the diagnosis of breast cancer.

Physical examination and mammography are currently the only proven and reliable methods of early detection of breast cancer. Although both procedures are highly sensitive, their limited specificity often requires surgical biopsy in order to differentiate between malignant and benign lesions. The purpose of this prospective study is to investigate the diagnostic specificity of thallium imaging for breast cancer and to determine its efficacy as a complement to mammography. Two groups were studied: Group A: Patients found to have breast abnormalities and scheduled for biopsy or surgery and Group B: Patients who were suspected to have a recurrence of cancer after mastectomies or lumpectomies. In Group A, thallium scans of 32 breasts in 30 patients were performed prior to biopsy or surgery, yielding pathological diagnoses of 31 breasts in 29 patients. Results for Group A included seven true-positive thallium scans, twenty-two true-negative scans, two false-negative scans, and one false-positive scan. In Group B, seven patients were scanned to evaluate subcutaneous nodules for breast cancer following mastectomy or lumpectomy. Results for Group B included five true-positive scans, one true-negative scan, one false-negative scan and no false-positive scans. Thallium breast scanning was shown to have high specificity for cancer (specificity 96% and sensitivity 80%), suggesting that this technique should be evaluated in additional patient studies to determine its role in clinical situations.

Cytomegalovirus pseudotumor presenting as bowel obstruction in a patient with acquired immunodeficiency syndrome.

Although cytomegalovirus (CMV) can be fatal to patients with the acquired immunodeficiency syndrome (AIDS), it usually causes few, if any, symptoms. The virus has an affinity for the alimentary tract, especially the ileum and right colon. CMV infections of the gut are often erosive, resulting in enterocolitis, hemorrhage, or intestinal perforation. Inflammatory mass formation is rare. Kaposi's sarcoma and lymphoma are established causes of bowel obstruction in patients with AIDS. This report describes a case of ileocecal obstruction due to a discrete CMV-induced pseudotumor in a patient with AIDS.

Novel approach to iatrogenic bile peritonitis.

Bile peritonitis after injury to the biliary tree is a serious complication that requires exploratory laparotomy. Our patient had an obstructing ampullary carcinoma, and generalized bile peritonitis developed from attempted percutaneous transhepatic cholangiography. The patient's condition was managed by peritoneal lavage and endoscopic transampullary stenting, with immediate relief of pain and toxicity. Exploratory laparotomy was avoided, and an eventual pylorus-sparing Whipple resection was the definitive treatment. We believe this to be the first report of successful nonoperative treatment of a patient with bile peritonitis with obstructive jaundice.

Traumatic hemoperitoneum due to a Meckel's mesodiverticular band: case report.

The blood supply to a Meckel's diverticulum exists either within the small bowel mesentery, or within a separate mesodiverticular band. The latter is associated with bowel obstruction. It is a rare source of hemorrhage. The case report describes hemoperitoneum resulting from blunt trauma-induced disruption of a mesodiverticular band.