Successful treatment of systemic capillary leak syndrome with intravenous immunoglobulins

Almagro, P.; Martí, J.M.; Garcia Pascual, L.; Rodriguez-Carballeira, M.

doi:10.1016/j.rce.2011.08.004

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"apellidos" => "Almagro" "email" => array:1 [ 0 => "19908pam@comb.cat" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "¿" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "J.M." "apellidos" => "Martí" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "L." "apellidos" => "Garcia Pascual" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "c" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "M." "apellidos" => "Rodriguez-Carballeira" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Internal Medicine, University Hospital Mútua de Terrassa, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Oncohematology, University Hospital Mútua de Terrassa, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Endocrinology Services, University Hospital Mútua de Terrassa, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tratamiento eficaz del síndrome de extravasación capilar sistémica con inmunoglobulinas intravenosas" ] ] "textoCompleto" => "Systemic capillary leak syndrome (SCLCS) or Clarkson's Syndrome is a rare and life-threatening disorder characterized by repeated episodes of severe hypotension, hypoalbuminemia and hemoconcentration. Attacks are characterized by marked shifts of plasma and proteins to the extravascular space. Mortality during episodes is high (by persistence of shock or pulmonary oedema to reverse the syndrome, in the recruitment phase). In almost every case reported, a monoclonal immunoglobulin has been present in the serum, but its role in the disease has not been clarified.1We present a patient affected by this rare entity, with a prolonged survival in which the crises have disappeared temporarily after treatment with melphalan–prednisone on two occasions, and lately with intravenous immunoglobulins (IVIG).A 48-year-old woman was first admitted to hospital in 1997 with severe hypotension, hemoconcentration (hemoglobin 21g/l) and hypoalbuminemia (23g/l). The patient was managed in the Intensive Care Unit (ICU) with volume and dopamine; she remained hypotensive for 48h, oligoanuric and with progressive oedema. She finally recovered her blood pressure with progressive polyuria, disappearance of oedema and normalization of hemoglobin and albumin. A serum monoclonal band of Ig Kappa of 3.5g/L was documented, with a normal bone marrow examination. She was found to be hypothyroid, with a thyrotropin of 69ng/dL (normal values: 0.8–1.8), thyroxine of 5.3pmol/L (normal values: 10.29–23.16) and positive antimicrosomal, antiperoxidase and antithyreoglobulin antibodies.The patient was diagnosed with SCLCS and treated with therbutaline and aminophylline. During follow-up to year 2000 she presented 20 similar episodes. In 2001, given the lack of therapeutic response, and after a normal new bone marrow biopsy, she was treated with melphalan/prednisone monthly for a year. With this treatment, the frequency and intensity of the episodes diminished until their total disappearance. In 2005 she relapsed with 6 new episodes. Serum levels of chromogranin A were found to be high (188ng/mL), and did not change during the crises, with a NTF-α that remained normal. An abdominal computerized tomography, nuclear magnetic resonance of the adrenal glands and scintigraphy with octeotride In111 and with Iodine123 metaiodobenzylguanidine were all within normal limits. After a repeat bone marrow biopsy that was unrevealing, she restarted treatment with melphalan/prednisone monthly for a year. The patient remained newly asymptomatic for the next 24 months. The monoclonal band was greatly diminished (0.19 G/) and levels of chromogranin A remained normal (60ng/mL). Finally, in 2008 the patient presented 2 new episodes of severe shock which reversed promptly with IVIG. Because the patient had a refractory SCLS we finally decided to administer intravenous immunoglobulin (2g/kg) every 6 weeks. Since the beginning of this treatment, the patient has had complete resolution of recurrent episodes and has been asymptomatic up to the present.SCLS are characterized by recurrent crises of massive extravasation of plasma and its constituents to extravascular space as a result of a sudden reversible increase in capillary permeability. About 120 cases have been described, but recurrent episodes of shock were found in only 40 patients.<a class="elsevierStyleCrossRef" href="#bib0005">1</a> Capillary permeability is normal in quiescent periods. Usually no myeloma has been documented, although in four cases myeloma appeared during follow-up.<a class="elsevierStyleCrossRef" href="#bib0010">2</a> No curative treatment is available for SCLS, although different regimens have been tried with variable success. The most frequently used is the combination of terbutaline and aminophylline,<a class="elsevierStyleCrossRefs" href="#bib0005">1,3</a> which was unsuccessful in our patient. Interestingly, in patients who developed myeloma during their evolution, treatment with chemotherapy is accompanied by a disappearance or decrease of the attacks.<a class="elsevierStyleCrossRef" href="#bib0015">3</a> Based on these results some authors have proposed the use of chemotherapy in patients with repeated attacks.<a class="elsevierStyleCrossRef" href="#bib0015">3</a> However to the best of our knowledge, the patient reported here is the first in whom this regimen has proved effective, although only temporarily. More recently, Lambert et al. described a patient with scleroderma and chronic refractory SCLS in whom the monthly administration of IGIV was followed by disappearance of symptoms, and two other patients with SLCS were also controlled with the same regimen during the acute phase of the disease.<a class="elsevierStyleCrossRef" href="#bib0020">4</a> At least one other patient with repeated episodes of SCLCS has remained stable for three years with chronic administration of IGIV.<a class="elsevierStyleCrossRef" href="#bib0025">5</a> More recently Gousseff et al.<a class="elsevierStyleCrossRef" href="#bib0030">6</a> had reported a similar data in other 18 patients recruited in a European Register. In conclusion, our observation suggests for the first time that there is a beneficial effect from therapy with melphalan–prednisone, which is reversible with the finalization of treatment, and strengthens the hypothesis of the action of intravenous immunoglobulins." 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