Clinical disorders responsible for plasma hyperviscosity and skin complications

Caimi, Gregorio; Carlisi, Melania; Urso, Caterina; Presti, Rosalia Lo; Hopps, Eugenia

doi:10.1016/j.ejim.2017.04.001

Cited by 8 publications

(7 citation statements)

References 83 publications

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“…In severe cases, immunosuppressive therapy, plasmapheresis, and intravenous fibrinolysis have been recommended. 25,30,37 Nevertheless, although clinical manifestations of cryofibrinogenemia can resolve and CF in plasma can disappear, the persistence of CF in plasma up to 20 months later has also been reported in some cases. 29 Our experience with this study might suggest that CF should also be evaluated in idiopathic chilblains and in those associated with autoinflammatory disorders, such as type 1 interferonopathies, as well as monogenic familiar chilblain lupus.…”

Section: Discussionmentioning

confidence: 99%

“…Stanozolol is also an effective therapeutic alternative. In severe cases, immunosuppressive therapy, plasmapheresis, and intravenous fibrinolysis have been recommended 25,30,37 . Nevertheless, although clinical manifestations of cryofibrinogenemia can resolve and CF in plasma can disappear, the persistence of CF in plasma up to 20 months later has also been reported in some cases 29 …”

Section: Discussionmentioning

confidence: 99%

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High prevalence of cryofibrinogenemia in patients with chilblains during the COVID‐19 outbreak

et al. 2020

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Background Many cutaneous manifestations have been described in possible association with the COVID-19 pandemic, including acral lesions resembling chilblains. The underlying pathomechanisms of COVID-19 chilblains are not fully understood. The aim of this study was to describe the clinical, pathological, and laboratory findings of a series of patients who developed chilblains during the COVID-19 outbreak and to investigate the possible factors that could be involved in the pathogenesis of these lesions. Methods We conducted a prospective cohort study that included 54 patients who presented with chilblains during the highest peak in the incidence of COVID-19 in Cantabria (northern Spain). Skin biopsies were performed on 10 of these patients who presented with recent lesions. Laboratory investigations, including immunological analysis, serological studies, and the assessment of cryoproteins, were also performed. Results Most patients presented erythematous plaques located on the toes and/or purpuric macules located on the feet. Histopathological findings were compatible with those of idiopathic chilblains. Immunohistochemical evaluation showed C3d and C4d deposits in the vessel walls in seven cases. The autoimmunity panel was negative in most of our series. Cryoprotein testing showed positive cryofibrinogen in two-thirds (66.7%) of the patients assessed. On follow-up, most patients presented almost complete resolution, although six patients required prednisone and antiaggregant drug treatment. Conclusions This study shows, for the first time to our knowledge, a high prevalence of cryofibrinogenemia in patients with chilblains during the COVID-19 pandemic. Cryofibrinogenemia could be implicated in the pathogenesis of chilblains related to COVID-19.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

High prevalence of cryofibrinogenemia in patients with chilblains during the COVID‐19 outbreak

et al. 2020

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“…[ 2 ] In type I cryoglobulinemia, a predominant role for hyperviscosity in the pathogenesis of vasculitis cutaneous manifestations has been proposed, with minor relevance of immune complex-induced vasculopathy. [ 10 ] However, in our patient, as in the majority of reported cases of organ damage due to monoclonal gammopathy-associated type I cryoglobulinemia, [ 2 , 3 , 8 , 9 ] no classical manifestations of hyperviscosity syndrome were found.…”

Section: Discussionmentioning

confidence: 47%

A multiple myeloma that progressed as type I cryoglobulinemia with skin ulcers and foot necrosis

Solimando

Sportelli

Troiano³

et al. 2018

Medicine

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Rationale:Type I cryoglobulinemia is a rare and life-threatening condition. It occurs mainly in B-cell lymphoproliferative disorder. In almost half of the patients, type I cryoglobulinemia is characterized by severe cutaneous involvement.Patient concerns:A 71-year-old man comes to our observation because of the onset of persistence of paresthesia and painful acrocyanosis in the fingers and toes. IgG-k multiple myeloma treated with chemotherapy and radiation therapy on the left iliac wing characterized his clinical history. At the evaluation of response after the first-line therapy, the patient achieved a very good partial response. At the time of progression, the same myelomatous disease has taken a typical behavior of cryoglobulinemia.Diagnoses:Type I multiple myeloma-associated cryoglobulinemia was diagnosed.Interventions:The patient underwent to an immediate composite therapeutic approach based on prostanoid infusion, plasmapheresis along with second-line chemotherapy.Outcomes:Despite the rapid biochemical response, the ischemia of the feet worsened. Moreover, a bacterial infection overlapped. The surgical amputation of both feet was necessary. Allowing MM cytoreduction continuation the patient's clinical condition became stabilized.Lessons:Though rare, type I cryoglobulinemia can be associated with plasma cell dyscrasias. Any delay in diagnosis and the start of therapy can cause worsening of organ damage and endanger the patient's life. Therapeutic strategies in these cases should be directed to the underlying diseases.

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“…1,5 The diagnosis is based in the results of bone marrow biopsy and serum protein electrophoresis with immunofixation. 5,6 HVS is found in 10 to 30% of patients with WM and can be the initial manifestation. 2,3 IgM is a large molecular structure secreted as a pentamer and is found mainly in the intravascular structures.…”

Section: Discussionmentioning

confidence: 99%

Hyperviscosity syndrome - a case report

Costa¹,

Almeida²,

Batista³

et al. 2019

IJFCM

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Hyperviscosity syndrome (HVS) is a life-threatening complication. The clinical manifestations include a variety of symptoms like visual symptoms, altered mental status, stroke or congestive heart failure. Prompt treatment is needed to avoid progression to multisystem organ failure. We report a case of a 73-year-old man with a 3-months history of headaches and altered mental status. His neurological exam showed symmetrical decreased pain, thermal and tactile sensitivity in the upper and lower limbs and symmetrical decreased muscle strength in the lower limbs. His eye exam showed retinal hemorrhages and dilated retinal veins. His blood counts showed anemia, increased C-reactive protein, sedimentation rate and serum viscosity. He had an elevated immunoglobulin M and serum immunofixation revealed Ig M-kappa paraprotein. The bone marrow aspiration and biopsy revealed a Waldenström Macroglobulinemia. He was diagnosed with HVS and was treated with plasmapheresis, chemotherapy and fluids.HVS diagnosis requires a high degree of clinical suspicion, andclinicians should be aware of suggestive clinical and laboratory findings.

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Clinical disorders responsible for plasma hyperviscosity and skin complications

Cited by 8 publications

References 83 publications

High prevalence of cryofibrinogenemia in patients with chilblains during the COVID‐19 outbreak

High prevalence of cryofibrinogenemia in patients with chilblains during the COVID‐19 outbreak

A multiple myeloma that progressed as type I cryoglobulinemia with skin ulcers and foot necrosis

Hyperviscosity syndrome - a case report

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