Type I cryoglobulinemia in multiple myeloma, a rare entity: analysis of clinical and biological characteristics of seven cases and review of the literature
Judith PayetJ. LivartowskiNiloufar KavianOlivia ChandesrisN. DupinNadège WalletAlexandre KarrasCarine SalliotFelipe SuárezHervé Avet‐LoiseauMarie Alexandra AlyanakianChadi Al NawakilSophie ParkJérôme TamburiniC. RouxDidier BouscaryL. Sparsa
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Abstract:
The type I cryoglobulins (CGs) account for 10–15% of all cryoglobulins and are found in patients with hematological disorders. We here describe the largest series of seven cases of type I cryoglobulinemia associated with multiple myeloma (MM) and provide a detailed review of the literature associated with this disorder, with the aim of improving the future diagnosis and therapeutic management of this rare disease. Six of the cases in our series were men aged 28–69 years, and most of the subject patients had an immunoglobulin G (IgG) monoclonal component and stage I indolent MM that manifested as cryoglobulin-related symptoms. The patients were all karyotypically normal. Clinical manifestations in this group were: skin lesions (five cases, 71.4%), rheumatologic failure (four cases, 57.1%), neurological abnormalities (two cases, 28.6%), mixed cutaneous/rheumatologic/renal defects (one case, 14.3%) and one case in which the cryoglobulinemia was asymptomatic. Two patients experienced acute renal failure but underwent a full recovery following treatment for MM. We conclude from our analysis that treatment approaches for severe type I cryoglobulinemia should involve plasmapheresis at the onset to achieve a rapid control of the CG-related symptoms, and that specific MM treatments should be introduced also at an early stage to avoid cryoglobulinemia relapse. In this context, bortezomib and lenalidomide are potentially the most effective therapeutic agents.Keywords:
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The management of patients with cryoglobulins often meets with limited success. Reported here is the use of plasmapheresis and/or partial plasma exchange in the management of five patients with cryoglobulinemia. The procedure was carried out at room temperature with reinfusion through a blood warmer. Circulating levels of mixed cryoglobulins and monoclonal IgM cryoglobulins were more easily reduced than were IgG cryoproteins. Improvement in symptoms was associated with removal of the cryoprotein. Pheresis can be used as primary therapy for reduction of cryoglobulin levels in cases of symptomatic essential cryoglobulinemia. Where an etiology for cryoglobulinemia is known and specific treatment exists, pheresis can be used as effective adjunct therapy.
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Case Reports1 August 1953CRYOGLOBULINEMIA: REPORT OF A CASE ASSOCIATED WITH GANGRENE OF THE DIGITSJAMES H. HUTCHINSON, M.D., ROY A. HOWELL, M.D.JAMES H. HUTCHINSON, M.D.Search for more papers by this author, ROY A. HOWELL, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-39-2-350 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptIn 1947 Lerner and Watson1 suggested the term "cryoglobulinemia" to describe the presence of a cold precipitable protein in the serum. Three years later Barr, Reader and Wheeler2 reviewed the literature and found eight cases in which significant amounts of cryoglobulin had been observed; to these they added two of their own. Three additional cases have recently been recorded.3, 4, 5In the case reported here, cold precipitable globulins were demonstrated in association with Raynaud's phenomena which progressed to gangrene and required multiple amputations. To our knowledge this is the first instance in which gangrene has been associated with cryoglobulinemia....Bibliography1. LernerWatson ABCJ: Studies of cryoglobulins. I. Unusual purpura associated with the presence of a high concentration of cryoglobulin (cold precipitable serum globulin), Am. J. M. Sc. 214: 410, 1947. CrossrefMedlineGoogle Scholar2. BarrReaderWheeler DPGCCH: Cryoglobulinemia. I. Report of two cases with discussion of clinical manifestations, incidence and significance, Ann. Int. Med. 32: 6, 1950. LinkGoogle Scholar3. Rorvik K: Cryoglobulinemia, Acta med. Scandinav. 137: 390, 1950. CrossrefMedlineGoogle Scholar4. LuceyLeighHochMarrackJohns HCEHJRRG: Study of a case of purpura associated with bone changes and formation of a gel in the serum on cooling, Brit. J. Exper. Path. 31: 380, 1950. MedlineGoogle Scholar5. BianchiGiampalmoMarmont VAA: Contributo alla conoscenza della gelificazione plasmatica a frigore e della plasmocitosi aleucemica con emogelificazione (gel-plasmocitomatosi), Minerva med. 2: 101, 1949. Google Scholar6. MuirheadMontgomeryGordon EEPOCE: Thromboembolic pulmonary vascular sclerosis: report of a case following pregnancy and of a case associated with cryoglobulinemia, Arch. Int. Med. 89: 41, 1952. CrossrefGoogle Scholar7. AllenBarkerHines EVWEA: Peripheral vascular disease, 1946, W. B. Saunders Co., Philadelphia, p. 195. Google Scholar8. BayrdHeck EDFJ: Multiple myeloma—a review of 83 proven cases, J. A. M. A. 133: 147, 1947. CrossrefMedlineGoogle Scholar9. MostLavietes HPH: Kala azar in American military personnel, Medicine 26: 221, 1947. CrossrefMedlineGoogle Scholar10. LernerBarnumWatson ABCPCJ: Studies of cryoglobulins. II. The spontaneous precipitation of protein from serum at 5° C. in various disease states, Am. J. M. Sc. 214: 416, 1947. CrossrefMedlineGoogle Scholar11. Forbes GB: Autohaemagglutination and Raynaud's phenomenon, Brit. M. J. 1: 598, 1947. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: *Received for publication January 2, 1953.From the Department of Internal Medicine, Washington University, School of Medicine, and the Unit I (Washington University) Medical Service, St. Louis City Hospital, St. Louis, Missouri. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byCerebral Ischemia in Patients With Hepatitis C Virus Infection and Mixed CryoglobulinemiaBilateral forefoot ischemia as a premonitory symptom of mixed cryoglobulinemiaDeafness in CryoglobulinemiaMixed cryoglobulinemia: Clinical aspects and long-term follow-up of 40 patientsEncephalopathy associated with cryoprecipitable Australia antigenBiologic and clinical significance of cryoglobulinsParaproteinämische HämoblastosenLupus ErythematosusNeurological Manifestations Associated with Cold Hemagglutinin Syndrome Report of Two CasesRetikulosenHemopoietic SystemCRYOPROTEINEMIA IN LEPROSYTissue hypoxia in surgical practiceRetinal Findings Associated with Serum HyperviscosityCirculatory Problems in DysproteinemiaMesothelioma associated with cryoglobulinemiaExperiments on the Mechanism of Precipitation of CryoglobulinsESSENTIAL CRYOGLOBULINAEMIA.Krankheiten der GefäßePlasma-Cell Myeloma"MYELOMA" SERUM ELECTROPHORETIC PATTERNS IN CONDITIONS OTHER THAN MYELOMATOSISDie Pathomorphologie der NierenausscheidungDysproteinemiaDie MakroglobulinämieCRYOGLOBULINAEMIAEssential cryoglobulinaemiaCryo- and macroglobulinemiaKryoglobulinämieMakromolekulare Kryoglobulin�mie 1 August 1953Volume 39, Issue 2Page: 350-357KeywordsChemical fractionationGlobulinsHospital medicineMedical servicesProteinsResearch laboratoriesSerum proteins Issue Published: 1 August 1953 PDF downloadLoading ...
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Cryoglobulinemia can be among the causes of sperm bad quality. But conventional examination algorithm for patients with idiopathic oligozoospermia makes no provision for cryoglobulin determination in blood serum.We examined 55 patients with idiopathic spermatogenesis disorder. Each patient had cryoglobulin determined in blood serum. For this purpose, optical density of the patient's blood serum before and after its 7-day cooling at a temperature of 4°C was compared. Type of cryoglobulins was established by the method evaluation of serum optic density in different periods of cooling incubation (before and after cooling) using the curves of temperature resistance for comparison. Patients with cryoglobulinemia underwent intracutaneous immunization with autoleukocytes separated from heparinized venous blood.Cryoglobulins were revealed in 16 patients of all 55 examined (29.09%): in six patients' cryoglobulins of second type; in nine - third type and in one patient - cryoglobulins of first type were detected. In a control group, which consisted of 50 men is blood - donors' frequency of cryoglobulinemia was 2%. Pathogenetic connection between cryoglobulinemia and disturbances in sperm quality is also supported by the fact that after autoleukocyte immunization in patients who positively responded to cryoglobulinemia treatment (14 persons of 16 or 87.5%) spermogram was found to be improved. Thus, in 12 patient number of spermatozoa. In all patients progressive motility and precentage of normal forms increased with spermatoza concentration.It is reasonable to include determination of cryoglobulins in blood serum for patients with idiopathic oligo- and zoospermia.
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We report the case of a woman with long-lasting mixed cryoglobulinemic syndrome, who experienced clinical and laboratory remission of her cryoglobulinemia after becoming infected with human immuno-deficiency virus and developing HIV-1 induced immunosuppression.Serum cryoglobulin concentrations and the CD4+ cell count were monitored every three months.After the diagnosis of HIV-1 infection, the immunological status of the patient was constantly depressed (CD4+ cell count dropping from 337/microL in January 1991 to 21/microL in June 1994). Serum cryoglobulins were persistently absent over 43 months of follow-up, despite the presence of HCV-RNA.In this case, HIV-1 induced immunodeficiency seems to be responsible for the remission of the cryoglobulin syndrome and the disappearance of serum cryoglobulins. These findings indicate that CD4+ T lymphocytes may play a role in regulating the activity of cryoprecipitating rheumatoid factor secreting B cell clones.
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Cryoglobulins are immunoglobulins that precipitate in serum at temperatures below 37°C and resolubilize upon warming. The clinical syndrome of cryoglobulinemia usually includes purpura, weakness, and arthralgia, but the underlying disease may also contribute other symptoms. Blood samples for cryoglobulin are collected, transported, clotted and spun at 37°C, before the precipitate is allowed to form when serum is stored at 4°C in a Wintrobe tube for at least seven days. The most critical and confounding factor affecting the cryoglobulin test is when the preanalytical phase is not fully completed at 37°C. The easiest way to quantify cryoglobulins is the cryocrit estimate. However, this approach has low accuracy and sensitivity. Furthermore, the precipitate should be resolubilized by warming to confirm that it is truly formed of cryoglobulins. The characterization of cryoglobulins requires the precipitate is several times washed, before performing immunofixation, a technique by which cryoglobulins can be classified depending on the characteristics of the detected immunoglobulins. These features imply a pathogenic role of these molecules which are consequently associated with a wide range of symptoms and manifestations. According to the Brouet classification, Cryoglobulins are grouped into three types by the immunochemical properties of immunoglobulins in the cryoprecipitate. The aim of this paper is to review the major aspects of cryoglobulinemia and the laboratory techniques used to detect and characterize cryoglobulins, taking into consideration the presence and consequences of cryoglobulinemia in Hepatitis C Virus (HCV) infection.
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• An 80-year-old woman with essential cryoglobulinemia developed recurrent, widespread, purpuric lesions with superficial skin ulcers and cold urticaria. No disorders of other organ systems were recognized. Attempts to transfer the cold sensitivity passively by serum and isolated cryoprecipitate of the patient were successful. Immunochemical studies showed that the cryoglobulin was composed of IgG only. It was suggested that the cryoprecipitate might not be due to immune complex formation in this case. (Arch Dermatol114:784-786, 1978)
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