Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma

Abstract

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by third spacing of plasma into the extravascular compartment, leading to anasarca, hemoconcentration, and hypovolemic shock. It has been rarely associated with lymphomas, and reports usually indicate that it occurs after antineoplastic treatment. We present the case of a patient with ALK-negative anaplastic large cell lymphoma who presented with SCLS as the initial manifestation of her lymphoma. The SCLS resolved with treatment of the malignancy with steroids and chemotherapy.

Plasma filtration with dialysis for the treatment of capillary leak syndrome occurring secondary to surgery for colon cancer‐related perforating peritonitis

“…Other differential diseases, such as ovarian hyper‐stimulation syndrome and hereditary angioedema, were ruled out based on serum estradiol level and C1 esterase inhibitor level, respectively. Although there have been reports of CLS complicated with cancer, 7 the relationship between cancer and CLS is unclear. In addition, CLS occurring with myasthenia gravis has not been reported in the past, and it is unclear whether the two diseases are related.…”

“…1 The characteristics of CLS are hypotension, hemoconcentration, and low plasma albumin levels; however, there are no clear diagnostic criteria. [2][3][4][5][6][7][8] Certain autoimmune systems may be involved in the disease. Various treatments have been attempted, including fluid resuscitation, renal replacement therapy, corticosteroid administration, high-dose intravenous immunoglobulin (IVIG), and plasma exchange (PE).…”

“…Capillary leak syndrome (CLS) is a very serious condition of unknown cause, first reported by Clarkson in 1960 1 . The characteristics of CLS are hypotension, hemoconcentration, and low plasma albumin levels; however, there are no clear diagnostic criteria 2‐8 . Certain autoimmune systems may be involved in the disease.…”

Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

“…Because of the prominence of monoclonal gammopathy in SCLS melphalan and prednisolone have been tried to reduce immunoglobulin load with little effect 7 8. Although steroids have been used to maintain remission in the chronic form of SCLS there is limited evidence of effect in the acute setting although methylprednisolone has been used in SCLS associated with anaplastic lymphoma with good effect9 and prednisolone 1 mg/kg appeared to lead to an improvement in symptoms where plasmaphoresis and IVIG had not 10…”

Systemic capillary leak syndrome after surgery for colon cancer perforating peritonitis treated using plasma filtration with dialysis

“…Systemic capillary leak syndrome (SCLS) is a very serious condition of unknown cause, first reported by Clarkson in 1960 (1). The characteristics of SCLS are hypotension, hemoconcentration, and low plasma albumin levels; however, there are no clear diagnostic criteria (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). Certain autoimmune systems may be involved in the disease.…”