Idiopathic Systemic Capillary Leak Syndrome Preceding Diagnosis of Infiltrating Lobular Carcinoma of the Breast With Quiescence During Neoadjuvant Chemotherapy

Systemic capillary leak syndrome (SCLS) is a rare health condition. It is characterized by recurrent episodes of generalized edema and severe hypotension along with hypoproteinemia. The condition is under recognized because of its nonspecific signs and symptoms, and high mortality rate. SCLS triggered by trastuzumab, a target drug for Her2-positive breast cancer patients, has not been previously reported. A 59-year-old Chinese woman, diagnosed with breast cancer with accompanying liver and bone metastasis, was treated with 3 cycles of docetaxel with capecitabine and a regimen of 12 cycles of capecitabine with trastuzumab. The patient developed systemic capillary leak syndrome during the 16th cycle of chemotherapy. Post-diagnosis treatment regimen is also presented in the current case report. SCLS has been previously observed in breast cancer patients. However, SCLS incidence post-chemotherapeutic treatment with trastuzumab has not been reported elsewhere. Hence, our report highlights the need for rigorous investigation of the side effects of trastuzumab usage and the increasing need of insightful diagnosis to manage any incidence of SCLS. The case provides valuable experience for treating the uncommon adverse effects of trastuzumab in Her2-positive breast cancer patients.

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Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient

Zhang

Yang

2013

Pathol. Oncol. Res.

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Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

140

172

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In 1960, Dr. Bayard Clarkson described a woman experiencing sporadic, recurrent episodes of shock and anasarca. Plasma from an acute attack induced a “shock”-like syndrome when injected into rats. The enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is characterized by transient and severe, but reversible, hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules into tissues. Although < 500 cases of SCLS have been reported in the literature since 1960, the condition is probably under-diagnosed due to lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis since its presentation can resemble more common plasma leakage syndromes including angioedema or systemic anaphylaxis. Although the precise molecular etiology of SCLS remains unknown, substantial advances over the last five years have increased our understanding of SCLS pathogenesis.

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Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments

Anipindi¹,

Kacarow²,

Bitetto³

2023

Cureus

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Systemic capillary leak syndrome (SCLS) is due to increased capillary permeability to proteins and fluid extravasation from blood vessels into surrounding tissues and body cavities. This fluid extravasation leads to hypotension, generalized anasarca, pleural effusions, and pericardial effusions --the more severe cases of SCLS can cause multiorgan dysfunction, including cardiovascular collapse, shock, and death. The treatment includes corticosteroids, diuretics, albumin, immunoglobulins, and crystalloids. SCLS is potentially fatal. Recognizing signs and symptoms early and treating the patients is essential as this condition is fatal. It sometimes is a diagnosis of exclusion, being very challenging to diagnose and treat. The lack of understanding of the underlying mechanisms causing SCLS and proper treatment guidelines, especially in cancer patients, made diagnosing and treating this condition hard. Reports show that many cancers and anti-cancer treatments, including newer immunotherapy, cause SCLS. The mortality rate of SCLS associated with cytotoxic chemotherapy is 24% at five years. This review focuses on the cancers and anti-cancer drugs causing SCLS, treating acute SCLS, and available preventive regimens. The fundamental purpose of this review is to help clinicians recognize SCLS early to avoid delays in diagnosis and treatment. We also would like to elaborate on the fact that research on cancer-related SCLS is critical for developing staging criteria, useful diagnostic markers, prevention, and treatment strategies for anti-cancer drug-induced SCLS to prevent early discontinuation of anti-cancer drugs.

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Idiopathic Systemic Capillary Leak Syndrome Preceding Diagnosis of Infiltrating Lobular Carcinoma of the Breast With Quiescence During Neoadjuvant Chemotherapy

Cited by 3 publications

References 32 publications

Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient

Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments

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