Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature

Mullane, Ryan; Langewisch, Eric; Florescu, M; Plumb, Troy J.

doi:10.5414/cn109467

Cited by 12 publications

(7 citation statements)

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“…On the other hand the chronic form of ISCLS is extremely rare with only a few cases reported in the literature [5–8]. Unlike the classic form of ISCLS, chronic ISCLS is characterized by persistent edema with no signs of recovery and may be responsive to treatment with systemic steroids and IVIG [5, 7]. Similar to our case, monoclonal gammopathy is classically found in both forms of ISCLS.…”

Section: Discussionsupporting

confidence: 77%

Chronic idiopathic systemic capillary leak syndrome: a case report

Alkhunaizi¹,

Kabbani²,

ElTigani³

2019

Allergy Asthma Clin Immunol

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Background Idiopathic systemic capillary leak syndrome (ISCLS), is a rare disorder characterized by recurrent attacks of varying severity of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia in the absence of albuminuria. The chronic form of ISCLS is extremely rare with only a few cases reported in the literature. Case presentation Here we report the case of a young woman who developed chronic ISCLS characterized by massive, generalized and persistent edema. The work up confirmed the presence of monoclonal gammopathy. She was treated with several agents with no response. Conclusion Chronic ISCLS is a very rare disease of unknown etiology and results in devastating complications. The medical community should be aware of this disease with the hope that targeted therapy will become available in the future.

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Section: Discussionsupporting

confidence: 77%

Chronic idiopathic systemic capillary leak syndrome: a case report

Alkhunaizi¹,

Kabbani²,

ElTigani³

2019

Allergy Asthma Clin Immunol

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“…There are only a few case reports on the chronic form of SCLS [4,[10][11][12][13][14][15]. Unlike the classical form, patients with chronic SCLS experience mild progressive generalized edema, along with pleural and pericardial effusions without signs of spontaneous recovery.…”

Section: Discussionmentioning

confidence: 99%

“…Unlike the classical form, patients with chronic SCLS experience mild progressive generalized edema, along with pleural and pericardial effusions without signs of spontaneous recovery. Idiopathic chronic SCLS reportedly shows a therapeutic response to systemic steroids [10,15] or to intravenous immunoglobulin [12]. Moreover, chronic SCLS induced by gemcitabine has also been reported [13,14].…”

Section: Discussionmentioning

confidence: 99%

Chronic systemic capillary leak syndrome associated with an intravascular large B-cell lymphoma in a patient undergoing hemodialysis: a case report with literature review

et al. 2021

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Background Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by hypotension, hemoconcentration, and hypoalbuminemia associated with increased capillary endothelium permeability. Patients with a chronic form of SCLS present with persistent and progressive generalized edema. However, there have been no reports of chronic SCLS in patients undergoing hemodialysis. Herein, we report a case of chronic SCLS associated with an intravascular large B-cell lymphoma (IVLBCL) in a patient undergoing hemodialysis. Case presentation A 71-year-old male had been on hemodialysis for five years due to diabetic nephropathy. Difficulty in body fluid removal was observed during hemodialysis, and the patient was admitted to our hospital due to exacerbated weight gain and lower limb edema. He had elevated serum lactate dehydrogenase (LDH) levels and thrombocytopenia. His blood pressure was low, and his serum brain natriuretic peptide level was relatively low, despite the increase in body fluid volume. His clinical characteristics suggested a chronic form of SCLS. Random skin biopsy revealed IVLBCL; however, the association between IVLBCL and chronic SCLS remained unclear. He underwent chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone, followed by rituximab. After the treatment, his serum LDH level decreased, and the difficulty in body fluid removal during hemodialysis improved. The patient’s chronic SCLS seemed to be complicated by IVLBCL. Conclusions Patients with chronic SCLS who are undergoing hemodialysis seem to present with difficulties in fluid removal. The frequency of SCLS complications in cases with malignant lymphomas, including IVLBCL, is considered to be extremely low. However, clinicians should be aware of SCLS as a complication of malignant lymphomas.

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“…In the recovery period, patients' capillary permeability gradually returns to normal, manifesting as gradual subsidence of edema, decreased body weight, increased blood pressure and central venous pressure, and increased urine output. Mullane et al [17] reported that after active treatment, one patient had completely alleviated edema, reduced body weight, increased albumin, gradually disappearing symptoms, and continuously improving condition. In our study, all 47 patients in the CLS-sHLH group had clinical features of CLS at the early stage of sHLH.…”

Section: Discussionmentioning

confidence: 99%

Clinical features of 47 secondary hemophagocytic lymphohistiocytosis patients complicated with capillary leak syndrome

et al. 2020

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The clinical features of patients with secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) remain controversial. The data of 259 sHLH patients were retrospectively analyzed. The clinical manifestations, laboratory findings, treatment, and prognosis of the CLS-sHLH group and non-CLS-sHLH group were compared. The levels of fibrinogen, albumin, and serum calcium in the CLS-sHLH group were lower than in the non-CLS-sHLH group, and serum triglycerides in the CLS-sHLH group were higher than in the non-CLS-sHLH group (P < 0.05). Univariate analysis showed that fibrinogen level was an independent prognostic factor in sHLH patients complicated with CLS. The median survival time was significantly shorter in patients with fibrinogen ≤ 1.3 g/L than in patients with fibrinogen > 1.3 g/L (P < 0.05). Patients with improved CLS conditions in the CLS-sHLH group had significantly increased albumin and serum calcium after treatment (P < 0.05); patients without improved conditions in the CLS-sHLH group also had significantly increased albumin after treatment (P < 0.05), but the serum calcium did not change significantly (P > 0.05). sHLH patients complicated with CLS had significantly worse prognosis than without CLS. Significant reduction in fibrinogen may be an independent prognostic factor for poor prognosis in sHLH patients complicated with CLS.

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Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature

Cited by 12 publications

References 0 publications

Chronic idiopathic systemic capillary leak syndrome: a case report

Chronic idiopathic systemic capillary leak syndrome: a case report

Chronic systemic capillary leak syndrome associated with an intravascular large B-cell lymphoma in a patient undergoing hemodialysis: a case report with literature review

Clinical features of 47 secondary hemophagocytic lymphohistiocytosis patients complicated with capillary leak syndrome

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