CHEST 2013; 144 ( 1 ): 350 – 353
An 85-year-old man presented to an outside hospi- tal with 2 weeks of progressive vague chest discomfort and worsening shortness of breath. Upon presentation he was septic, with fever, tachypnea, and tachycardia. Chest radiograph showed a large sponta- neous left-sided pneumothorax with a large pleural effusion. A left chest tube was placed, and he was fluid resuscitated, placed on vasopressors and broad- spectrum antibiotics, and transferred to our institu- tion for further management. His past medical history was significant for early-stage chronic lymphocytic leukemia (CLL), which has been monitored but never treated, diabetes, gout, and atrial fibrillation.
Physical Examination Findings
Upon arrival at our hospital, he appeared malnour- ished and remained septic with a temperature as high as 103°F and systolic BP of , 90 mm Hg. Breath sounds were diminished on the left side. His chest tube drained 2 L of purulent fluid within the first few hours of presentation.
Gram stain from the pleural fluid showed gram- positive rods, gram-negative rods, and gram-positive cocci. Cultures grew out Staphylococcus aureus, Candida albicans, and Lactobacillus species. A CT scan of the chest (Fig 1) confirmed his left empyema and was concerning for a gastric-pleural fistula. An upper GI barium-swallow study confirmed a fistula between the gastric and pleural space (Fig 2). Intraoperatively, the stomach was notably inflamed and the proximal area was remarkable for a friable, necrotic-like mass, surrounded by fibrinous exudates and a clearly visible fistula tract.
The cause is: Transformation of CLL into diffuse large B-cell lymphoma, also known as Richter syndrome
Empyema is characterized by intrapleural pus usu- ally as a complication from a progressive parapneu- monic effusion. The development of an empyema has three distinct phases: (1) the exudative phase, with free-flowing and nonviscous fluid; (2) the fibrino- purulent phase, or more viscous fluid with intra- pleural loculations and pleural peels; and (3) the organizing phase, with fibrotic peels and loculations. Most commonly an empyema develops from similar pathogens that cause a pneumonia. A GPF, or a con- nection between the stomach and pleural cavity, is a rare cause of empyema. A GPF can arise from a per- forated gastric ulcer, esophageal or gastric surgery, trauma, empyema, and, in this case, invasive cancer.
Richter transformation denotes the development of high-grade non-Hodgkin’s lymphoma, prolympho- cytic leukemia (most common), Hodgkin’s lymphoma, or acute leukemia in patients with CLL and small lymphocytic leukemia. The first case series of Richter syndrome (RS) was reported in 1964. They described four cases of progression of CLL to “malignant retic- ulopathy,” with presentations that were similar to a case of rapidly progressing lymphadenopathy and hepa- tosplenomegaly in a patient with CLL first described by Maurice N. Richter in the 1920s. The incidence of RS has been reported between 2.2% and 8% and is gen- erally considered a rare disease. Since RS requires a pathologic assessment for diagnosis, the actual incidence might be higher than ones reported given the large heterogeneity in biopsy policies across institutions.
RS is a heterogeneous entity, which encompasses at least two different conditions: (1) transformation of CLL cells to a clonally related diffuse large B-cell lymphoma (DLBCL) (most common) and (2) devel- opment of a DLBCL that is unrelated to the CLL clone. The disease involves most frequently the lymph nodes, but extranodal localizations are also not uncommon and have been reported in the GI tract, skin, liver, tonsil, and bone marrow among other sites. Exact etiology of RS is unclear, but risk factors include trisomy 12, deletion of 11q23, micro- satellite instability, mutations in tumor suppressor genes, and Epstein-Barr virus infections. RS is usu- ally heralded by sudden clinical deterioration and a rapid increase in the size of a lymphoid mass at one site. Clinical features include systemic symp- toms (59%), progressive lymphadenopathy (64%), extranodal involvement (64%), elevation in lactate dehydrogenase (82%), and a monoclonal gammopa- thy (44%). The median time for the development of RS is 21.6 months.
GI involvement of RS is very rare. To date, a review of the literature of English language reveal 20 cases of extranodal GI involvement which include stomach, large and small bowel, liver, spleen, and adrenal gland. Of these, only eight had involvement of the stomach with DLBCL as the predominant pathology. This patient’s presentation of an empyema and pneumo- thorax together should be suspicious for a visceropleu- ral leakage and possible bronchopleural fistula. To the best of our knowledge, RS has not been described in the setting of a DLBCL causing a large left-sided GPF manifesting as an empyema.
Rituximab-CHOP (cyclophosphamide, hydroxydauno- rubicin, oncovin, prednisone) is considered standard therapy for de novo DLBCL and is currently used in many instances of RS, but results are not satisfactory. Studies on survival rates of GI involvement of RS are limited; however, a small case study revealed a median survival of 22 months.
Regarding our case, pathology samples from the gastric mass returned for DLBCL. The diagnosis of RS was made from the suspected conversion of the patient’s underlying CLL into DLBCL. The cause of the GPF was presumed secondary to the friable necrotic stomach mass later diagnosed as large B-cell lymphoma complicated by an empyema. Unfortunately, due to the poor functional status of our patient, the patient and family decided against aggressive therapy. He was discharged to home hospice for comfort care.
1. RS is a transformation of B-cell CLL into an aggressive diffuse B-cell lymphoma.
2. Although uncommon of itself, RS most often involves the lymph nodes, but extranodal localizations such as the GI tract, skin, liver, tonsil, and bone marrow have been reported.
3. In the setting of an empyema and pneumo- thorax, visceropleural leakage and bronchopleural fis- tula should be suspected.
4. Clinical suspicion of Richter transformation in a patient with known B-cell CLL is based on systemic systems, progressive lymphadenopathy, extranodal involvement, elevation in lactate dehydrogenase, and monoclonal gammopathy.
5. RS with GI involvement is extremely rare, and has never been reported in causing a GPF presenting as an empyema, prior to this case.
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