What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units.

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and frequently fatal disorder caused by an uncontrollable and ineffective systemic immune response. Patients initially present with fever, cytopenia, and hepatosplenomegaly, and subsequently develop multiorgan failure (MOF). Hemophagocytosis can be found on biopsy specimen but is not required. Acquired forms of HLH can occur in apparently healthy adults, while children present more often with an inherited form of the disease. Since HLH often presents with sepsis-like symptoms and organ dysfunction, patients are usually treated for presumed sepsis, which inevitably leads to delayed diagnosis and treatment. Intensivists need to have a low threshold for suspecting this disorder when previously healthy individuals present with a fulminant sepsis-like syndrome, which are unresponsive to conventional treatment. We present 3 patients with HLH who were admitted to our adult medical intensive care unit (MICU) over a 2-year period with fatal outcomes and emphasize the diagnostic importance of markedly elevated serum ferritin levels and the need for tissue biopsy in making an accurate diagnosis in a timely manner.

Evaluation and management of chronic pulmonary thromboembolic disease.

Pulmonary embolism (PE) is common and the majority of patients survive the acute event. Survivors are at increased risk for adverse outcomes, including persistent thrombi, recurrent embolism, chronic thromboembolic pulmonary hypertension (CTEPH), and death. Anticoagulation protects against recurrence, which has a high mortality rate. The recommended duration of anticoagulation for patients with reversible PE risk factors is 3 months. For patients with idiopathic PE or persistent risk factors, extended duration of anticoagulation is preferred, balanced with an individual patient's risk of hemorrhage, which in itself is a major cause of morbidity and mortality. Among patients with malignancy who develop venous thromboembolism (VTE), low-molecular-weight heparin is preferred over oral vitamin K antagonists in the first 6 months. Thereafter, anticoagulation should be continued indefinitely with either low-molecular-weight heparin or oral vitamin K antagonists. Inferior vena cava filters are not routinely recommended and should only be used in patients who have a contraindication to anticoagulation. Patients who have had VTE and with persistent or recurrent dyspnea should be evaluated for recurrence of VTE or development of CTEPH. Patients with recurrent VTE should be anticoagulated indefinitely. Routine screening for CTEPH in asymptomatic patients is not recommended. Echocardiography often provides the first indication of the presence of pulmonary hypertension. Once presence of CTEPH is established by right-sided heart catheterization and perfusion imaging (ie, ventilation/perfusion scintigraphy, computed tomography angiography, or pulmonary angiography), patients should be referred early to a center with expertise, as it is potentially surgically curable by pulmonary endarterectomy. Those who are deemed inoperable after being evaluated may gain symptomatic benefit from drugs approved for idiopathic pulmonary arterial hypertension. Lung transplantation may also be an option for patients who are not candidates for pulmonary endarterectomy.

The hospital-survival and prognostic factors of patients with solid tumors admitted to an ICU.

The decision to admit a patient with cancer to the intensive care unit (ICU) is complex. There are limited data as to the outcome and prognostic factors of patients with solid tumors admitted to the ICU. A retrospective chart review was undertaken to evaluate this issue. Over an 18-month period, 147 patients with solid tumors were admitted to our ICU. Lung, colorectal, and breast were the commonest sites of the primary tumors, with 52% of patients having metastatic disease. A total of 79 (54%) patients survived to hospital discharge, with 50 (34%) patients being discharged to home. Metastatic disease and the requirement for vasopressor agents were independent predictors of poor outcome. The relatively high survival rate of this cohort of patients should prompt a reevaluation of the ICU admission criteria for patients with solid tumors who become critically ill.

Clinical and pathologic manifestations of necrobiosis lipoidica-like skin involvement in sarcoidosis.

Necrobiosis lipoidica dibeticum (NLD) is a granulomatous skin disease mostly associated with diabetes mellitus. NLD has been reported in patients with other systemic disease. Also, the lesions of NLD may be clinically, and sometimes even histologically indistinguishable from other inflammatory skin lesions. We described three patients with established diagnosis of sarcoidosis that developed skin lesions consistent with NLD. The association of NLD-like skin lesion in sarcoidosis is not widely appreciated. The subject of NLD and sarcoidosis is reviewed.