Fatal Cryocrystalglobulinemia With Intravascular and Renal Tubular Crystalline Deposits

DeLyria, Paul A.; Avedschmidt, Sarah; Yamada, Chisa; Farkash, Evan A.

doi:10.1053/j.ajkd.2015.11.014

Cited by 25 publications

(24 citation statements)

References 13 publications

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“…However, serum tests for cryoglobulin are occasionally negative in patients with crystalglobulinemia, and cryoglobulin is not a sole cause of crystalglobulinemia. Nevertheless, the pathological consequences are similar in both diseases: multiple crystal thrombi develop in the capillaries and arterioles of systemic organs . However, lesions within large vessels have not been described in patients with either condition.…”

mentioning

confidence: 99%

“…Nevertheless, the pathological consequences are similar in both diseases: multiple crystal thrombi develop in the capillaries and arterioles of systemic organs. 5 However, lesions within large vessels have not been described in patients with either condition. We herein present a case of crystalglobulinemia involving laminar crystal deposition in the aorta and its first-degree tributaries.…”

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confidence: 99%

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Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Shintani-Domoto

Shinozaki‐Ushiku

Okuma

et al. 2017

Pathology International

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Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy and is characterized by crystal thrombi within systemic organs. We herein report the first described case of crystalglobulinemia accompanied by laminar crystal deposition in the large vessels. A 44-year-old man presented with a history of numbness, pain, and swelling of the left leg in addition to visual impairment. Renal and skin biopsies revealed crystal thrombi within the capillary lumens. The patient was finally diagnosed with crystalglobulinemia associated with multiple myeloma. He was treated with hemodialysis and chemotherapy but died of the disease 15 months after admission. Autopsy demonstrated a huge amount of crystal deposition in the subintimal layer of the vascular wall throughout the thoracic to abdominal aorta. The characteristic deposition extended to the iliac arteries, common carotid arteries, and subclavian arteries but did not affect the bilateral renal arteries. Antemortem computed tomography demonstrated higher intensity in the wall of the abdominal aorta but not in the walls of the renal arteries, suggesting that a finding of high intensity on computed tomography could be a clinical marker of systemic crystal deposition.

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confidence: 99%

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confidence: 99%

Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Shintani-Domoto

Shinozaki‐Ushiku

Okuma

et al. 2017

Pathology International

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“…15 Patients can present with acute kidney injury, renal artery thrombosis, skin rash/ulcerations, neurologic manifestations, neuropathy, polyarthritis, and blurry vision. [3][4][5][6][7][8][9][13][14][15][16][17][18] The latter symptom is a result of crystalline keratopathy that develops as a result of precipitation of these immunoglobulin crystals in the cornea. [3][4][5] Crystalline keratopathy has been reported in <1% of patients with myeloma-related disorders.…”

Section: Discussionmentioning

confidence: 99%

Crystalglobulin-Induced Nephropathy and Keratopathy

et al. 2019

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Crystalglobulinemia, a rare manifestation of monoclonal gammopathy, results from vascular deposition of crystallized monoclonal proteins leading to tissue injury. A 56-year-old man initially presented several years earlier with migratory polyarthralgias and blurry vision with no unifying diagnosis. Following an acute episode of malignant hypertension and rapidly progressive kidney failure, kidney biopsy was performed and was interpreted as idiopathic thrombotic microangiopathy. Further evaluation revealed an underlying monoclonal protein disorder. Slit-lamp biomicroscopy evaluation showed crystalline keratopathy. Re-evaluation of the kidney biopsy material with pronase staining confirmed crystalglobulin-induced nephropathy. The patient was initially treated with cyclophosphamide, bortezomib, and dexamethasone with partial response, followed by autologous stem cell transplantation with normalization of monoclonal protein studies, improvement in kidney function and joint symptoms, and decreased corneal deposits. His disease recurred but did not require additional treatment 1 year later. This case exemplifies the unique systemic presentation of diseases in the monoclonal gammopathy spectrum and emphasizes the need for a multidisciplinary approach when caring for these patients.

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“…Chemotherapy for plasma cell dyscrasia and plasmapheresis to remove pathogenic immunoglobulins are presumed to be beneficial for symptomatic relief. [2,3,5] Therapeutic strategies should be established to prevent fulminant end-organ damage caused by pathogenic M-proteins, including crystalglobulins.…”

Section: Discussionmentioning

confidence: 99%

“…[2] M-protein-related disease caused by pathological immunoglobulins and light chains, such as cryoglobulinemia, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome, AL amyloidosis, and light-chain deposition disease, can have diverse symptoms such as pyrexia, arthralgia, skin rash, peripheral neuropathy, thrombosis, and renal involvement, which mimic systemic rheumatic diseases. [3] Although M-protein-related diseases can cause fulminant end-organ damage, [4,5] indications for aggressive treatment of MGUS causing M-protein-related disease have not been known. We report a case of crystalglobulinemia, which is a rare form of M-protein-related disease, characterized by irreversible cryoprecipitation, and it is caused by monoclonal gammopathy that was previously diagnosed as MGUS.…”

Section: Introductionmentioning

confidence: 99%

Crystalglobulinemia manifesting as chronic arthralgia and acute limb ischemia

Abe

Tomita²,

Bohgaki³

et al. 2017

Medicine

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Rationale:Crystalglobulinemia is a rare disease caused by monoclonal immunoglobulins, characterized by irreversible crystallization on refrigeration. It causes systemic symptoms including purpura, arthralgia, and vessel occlusive conditions to be exacerbated by exposure to cold. We report a patient with crystalglobulinemia associated with monoclonal gammopathy of undetermined significance (MGUS) manifesting as chronic arthralgia and recurrent acute arterial occlusion.Presenting concerns:A 61-year-old man, who had been diagnosed with MGUS and who had arthralgia of unknown origin, presented with recurrent acute limb ischemia after surgical thromboembolectomy. Refrigeration of his serum formed precipitates that looked like needle-shaped crystals. These crystals did not dissolve with warming, which is not a characteristic of cryoglobulins. Skin biopsy results showed crystal-liked eosinophilic bodies in small vessels and we diagnosed crystalglobulinemia.Intervention and outcomes:Although he underwent above-knee amputation, he was treated with a bortezomib and dexamethasone-based chemotherapeutic regimen, following lenalidomide maintenance therapy. Finally, he achieved complete remission and serum crystalglobulins diminished.Lessons:Monoclonal gammopathy, previously diagnosed as MGUS, can cause systemic symptoms and thrombotic conditions by producing pathologic immunoglobulins, such as crystalglobulins. In such situations, MGUS, even when it has not progressed to multiple myeloma, can be a target of aggressive chemotherapy. Crystalglobulinemia should be considered for patients with monoclonal gammopathy manifesting as systemic and thrombotic symptoms exacerbated by cooling.

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Fatal Cryocrystalglobulinemia With Intravascular and Renal Tubular Crystalline Deposits

Cited by 25 publications

References 13 publications

Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Crystalglobulin-Induced Nephropathy and Keratopathy

Crystalglobulinemia manifesting as chronic arthralgia and acute limb ischemia

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