Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia

Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Martha Lacy, Morie Gertz, Francis K. Buadi, Suzanne R. Hayman, David M Dingli, Prashant Kapoor, Wilson Gonsalves, Ronald S. Go, Yi Lisa Hwa, Nelson Leung, Amie L. Fonder, Miriam A. Hobbs, Steven R. Zeldenrust, Stephen J Russell, John A. Lust, Robert A. Kyle, Shaji K Kumar

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n = 43, 42%) and ulcers/gangrene (n = 35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n = 24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients. An underlying lymphoproliferative disorder was identified in 94 (92%) subjects; MGUS—39, myeloma—20, lymphoplasmacytic lymphoma—21 and others—14. Treatment was initiated in 73 (72%) patients, primarily for cryoglobulinemia-related symptoms in 57. Treatment regimens consisted of: steroids ± alkylating agents in 29 (40%), novel myeloma therapies in 16 (22%), rituximab with alkylating agents in 12 (16%) and rituximab ± steroids in 11 (15%) patients; 22 patients received plasmapheresis. Six patients underwent autologous stem cell transplant. Cryocrit at treatment initiation, change in cryocrit and time to nadir cryocrit were predictive of symptom improvement. Treatment directed toward the underlying clonal disorder resulted in improvement (n = 47) or stabilization (n = 16) of symptoms in the majority of patients and disappearance of cryoglobulin in over one-half.

Original languageEnglish (US)
Pages (from-to)668-673
Number of pages6
JournalAmerican Journal of Hematology
Volume92
Issue number7
DOIs
StatePublished - Jul 1 2017

Fingerprint

Cryoglobulinemia
Alkylating Agents
Steroids
Cryoglobulins
Therapeutics
Raynaud Disease
Purpura
Gangrene
Plasmapheresis
Lymphoproliferative Disorders
Arthralgia
Glomerulonephritis
Ulcer
Stem Cells
Transplants
Kidney

ASJC Scopus subject areas

  • Hematology

Cite this

Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia. / Sidana, Surbhi; Rajkumar, S Vincent; Dispenzieri, Angela; Lacy, Martha; Gertz, Morie; Buadi, Francis K.; Hayman, Suzanne R.; Dingli, David M; Kapoor, Prashant; Gonsalves, Wilson; Go, Ronald S.; Hwa, Yi Lisa; Leung, Nelson; Fonder, Amie L.; Hobbs, Miriam A.; Zeldenrust, Steven R.; Russell, Stephen J; Lust, John A.; Kyle, Robert A.; Kumar, Shaji K.

In: American Journal of Hematology, Vol. 92, No. 7, 01.07.2017, p. 668-673.

Research output: Contribution to journalArticle

Sidana, Surbhi ; Rajkumar, S Vincent ; Dispenzieri, Angela ; Lacy, Martha ; Gertz, Morie ; Buadi, Francis K. ; Hayman, Suzanne R. ; Dingli, David M ; Kapoor, Prashant ; Gonsalves, Wilson ; Go, Ronald S. ; Hwa, Yi Lisa ; Leung, Nelson ; Fonder, Amie L. ; Hobbs, Miriam A. ; Zeldenrust, Steven R. ; Russell, Stephen J ; Lust, John A. ; Kyle, Robert A. ; Kumar, Shaji K. / Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia. In: American Journal of Hematology. 2017 ; Vol. 92, No. 7. pp. 668-673.
@article{669f491fd4b04c9ea349dba4ba24a0bb,
title = "Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia",
abstract = "We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87{\%}) patients, including: cutaneous symptoms in 64 (63{\%}) patients, with purpura (n = 43, 42{\%}) and ulcers/gangrene (n = 35, 34{\%}) being most common; neurological findings in 33 (32{\%}) patients, most frequently sensory neuropathy (n = 24, 24{\%}); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25{\%}); arthralgias in 24 (24{\%}); and renal manifestations, primarily glomerulonephritis in 14 (14{\%}) patients. An underlying lymphoproliferative disorder was identified in 94 (92{\%}) subjects; MGUS—39, myeloma—20, lymphoplasmacytic lymphoma—21 and others—14. Treatment was initiated in 73 (72{\%}) patients, primarily for cryoglobulinemia-related symptoms in 57. Treatment regimens consisted of: steroids ± alkylating agents in 29 (40{\%}), novel myeloma therapies in 16 (22{\%}), rituximab with alkylating agents in 12 (16{\%}) and rituximab ± steroids in 11 (15{\%}) patients; 22 patients received plasmapheresis. Six patients underwent autologous stem cell transplant. Cryocrit at treatment initiation, change in cryocrit and time to nadir cryocrit were predictive of symptom improvement. Treatment directed toward the underlying clonal disorder resulted in improvement (n = 47) or stabilization (n = 16) of symptoms in the majority of patients and disappearance of cryoglobulin in over one-half.",
author = "Surbhi Sidana and Rajkumar, {S Vincent} and Angela Dispenzieri and Martha Lacy and Morie Gertz and Buadi, {Francis K.} and Hayman, {Suzanne R.} and Dingli, {David M} and Prashant Kapoor and Wilson Gonsalves and Go, {Ronald S.} and Hwa, {Yi Lisa} and Nelson Leung and Fonder, {Amie L.} and Hobbs, {Miriam A.} and Zeldenrust, {Steven R.} and Russell, {Stephen J} and Lust, {John A.} and Kyle, {Robert A.} and Kumar, {Shaji K}",
year = "2017",
month = "7",
day = "1",
doi = "10.1002/ajh.24745",
language = "English (US)",
volume = "92",
pages = "668--673",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley-Liss Inc.",
number = "7",

}

TY - JOUR

T1 - Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia

AU - Sidana, Surbhi

AU - Rajkumar, S Vincent

AU - Dispenzieri, Angela

AU - Lacy, Martha

AU - Gertz, Morie

AU - Buadi, Francis K.

AU - Hayman, Suzanne R.

AU - Dingli, David M

AU - Kapoor, Prashant

AU - Gonsalves, Wilson

AU - Go, Ronald S.

AU - Hwa, Yi Lisa

AU - Leung, Nelson

AU - Fonder, Amie L.

AU - Hobbs, Miriam A.

AU - Zeldenrust, Steven R.

AU - Russell, Stephen J

AU - Lust, John A.

AU - Kyle, Robert A.

AU - Kumar, Shaji K

PY - 2017/7/1

Y1 - 2017/7/1

N2 - We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n = 43, 42%) and ulcers/gangrene (n = 35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n = 24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients. An underlying lymphoproliferative disorder was identified in 94 (92%) subjects; MGUS—39, myeloma—20, lymphoplasmacytic lymphoma—21 and others—14. Treatment was initiated in 73 (72%) patients, primarily for cryoglobulinemia-related symptoms in 57. Treatment regimens consisted of: steroids ± alkylating agents in 29 (40%), novel myeloma therapies in 16 (22%), rituximab with alkylating agents in 12 (16%) and rituximab ± steroids in 11 (15%) patients; 22 patients received plasmapheresis. Six patients underwent autologous stem cell transplant. Cryocrit at treatment initiation, change in cryocrit and time to nadir cryocrit were predictive of symptom improvement. Treatment directed toward the underlying clonal disorder resulted in improvement (n = 47) or stabilization (n = 16) of symptoms in the majority of patients and disappearance of cryoglobulin in over one-half.

AB - We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n = 43, 42%) and ulcers/gangrene (n = 35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n = 24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients. An underlying lymphoproliferative disorder was identified in 94 (92%) subjects; MGUS—39, myeloma—20, lymphoplasmacytic lymphoma—21 and others—14. Treatment was initiated in 73 (72%) patients, primarily for cryoglobulinemia-related symptoms in 57. Treatment regimens consisted of: steroids ± alkylating agents in 29 (40%), novel myeloma therapies in 16 (22%), rituximab with alkylating agents in 12 (16%) and rituximab ± steroids in 11 (15%) patients; 22 patients received plasmapheresis. Six patients underwent autologous stem cell transplant. Cryocrit at treatment initiation, change in cryocrit and time to nadir cryocrit were predictive of symptom improvement. Treatment directed toward the underlying clonal disorder resulted in improvement (n = 47) or stabilization (n = 16) of symptoms in the majority of patients and disappearance of cryoglobulin in over one-half.

UR - http://www.scopus.com/inward/record.url?scp=85019752232&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85019752232&partnerID=8YFLogxK

U2 - 10.1002/ajh.24745

DO - 10.1002/ajh.24745

M3 - Article

VL - 92

SP - 668

EP - 673

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 7

ER -