Presentation
We report two patients presenting with LCV who were found to have multiple myeloma on investigation. Multiple myeloma rarely presents as LCV. [1] However, the diagnosis must be suspected in a patient who does not respond to conventional therapy. [ijdvl.com]
This usually develops several years after the diagnosis of Waldenström macroglobulinaemia has been made, but can be the presenting problem. [dermnetnz.org]
Hematuria is the most common presenting sign, but is found in only 50-60% of cases. One-third of tumors are palpable at the time of diagnosis, and one-third present with abdominal or flank pain. [clinicaladvisor.com]
Peripheral blood eosinophilia and elevated IgE levels are often present. Circulating immune complexes, hypocomplementemia, and hyperglobulinemia have been observed but do not aid in the differential diagnosis. P-ANCA is occasionally present. [rheumaknowledgy.com]
Most of CM presented with clinical presentation of papules and nodules and was mainly due to the infiltration in dermis. [dovepress.com]
Entire Body System
- Severe Pain
May become chronic Unknown etiology 57 Deep Vein Thrombosis Almost always affects femoral vein Can cause reversible ischemia or frank gangrene Patients may develop, either abruptly or gradually, severe pain, extensive edema, and cyanosis of an extremity [slideplayer.com]
Hematological
- Easy Bruising
Lesions of colloid milium may become purpuric after trauma.27 FEMALE EASY-BRUISING SYNDROME The female predominance and the frequent association with phases of the menstrual cycle suggest that the female easy-bruising syndrome (purpura simplex) is due [medtextfree.wordpress.com]
bruising and spontaneous bleeding from the small vessels. [43] Frequently, the bleeding is mainly in the skin, causing petechiae, ecchymoses or both and in some cases there is bleeding also from the mucous membrane. [43] Vascular defect of hemostasis [ijdr.in]
Cardiovascular
- Heart Disease
Sore Throat, Urinary Retention, Valvular Heart Disease and Vegetative State. [books.google.com]
[…] wasting) Primary Idiopathic or genetic Transient (infants) Carbonic anhydrase deficiency, inhibition, or alteration Acetazolamide, sulfanilamides Carbonic anhydrase, type II deficiency Idiopathic Mafenide acetate York-Yendt syndrome Cyanotic congenital heart [clinicclinic2.cafe24.com]
Affected organs (such as the heart or lungs) may require specific medical treatment intended to improve their function during the active phase of the disease. [en.academic.ru]
Musculoskeletal
- Arthralgia
The classical presentation is “Meltzer’s triad” of palpable purpura, arthralgias and weakness (present in 25-30% and up to 80% at the disease onset). [clinicaladvisor.com]
[…] symmetric, distal, progressive, sensorimotor neuropathy, ataxic gait, bilateral foot drop IgM with other specificities: symmetric, distal, progressive painful sensory neuropathy prominent motor neuropathy IgG cryoglobulinemia type II weakness, purpura, arthralgias [ufrgs.br]
Joint involvement, particularly arthralgias and nondeforming arthritis, is common. [enotes.tripod.com]
This analysis also found arthralgia/arthritis to have negative association with anti-Ro and anti-La positivity, thereby, adding further strength to the findings of the cluster analysis. [openrheumatologyjournal.com]
These include severe constitutional symptoms, arthralgias, mononeuritis multiplex, and occasional cardiac or gastrointestinal symptoms. Lung and skin findings are more common in Churg-Strauss angiitis than in PAN. [rheumaknowledgy.com]
Skin
- Purpura
Curtz FR: Purpura hyperglobulinaemica, Ugesk. f. læger 108: 340, 1946. Google Scholar 3. Humerfelt S: Purpura with hyperglobulinemia, Nord. med. 34: 1101, 1947. MedlineGoogle Scholar 4. [acpjournals.org]
About a case of hyperglobulinemic purpura] [Article in Italian] V VANNUCCHI et al. Riv Crit Clin Med. 1954. No abstract available Similar articles [Hyperglobulinemic purpura]. WOLOSZCZUK I. WOLOSZCZUK I. Pol Tyg Lek. 1961 May 1;16:681-3. [pubmed.ncbi.nlm.nih.gov]
[…] of Waldenstrom Hyperglobulinemic Purpura Hyperglobulinemic Purpura of Waldenström Waldenstrom Hyperglobulinemic Purpura Hyperglobulinemic Purpura, Waldenstrom Hyperglobulinemic Purpuras Purpura, Waldenstrom Hyperglobulinemic Purpuras, Hyperglobulinemic [decs.bvs.br]
Purpura with Hyperglobulinemia, Nord. med. 34:1101, 1947. 8. Kay, H. E. M., and Robertson, K. M.: Benign Hyperglobulinaemic Purpura, J. Path. & Bact. 70:543, 1955. 9. Lindeboom, G. A.: Purpura Hyperglobulinemica, Dermatologica 96:337, 1948. 10. [deepdyve.com]
Secondary hyperglobulinemic purpura of Waldenström is characterized by polyclonal gammopathy associated mainly with autoimmune diseases. Its occurrence with multiple myeloma is very rare. [ncbi.nlm.nih.gov]
- Petechiae
Blood spots under the skin may be either purpura or petechiae. CD36 deficiency may be a cause of post transfusion purpura. People with Henoch's purpura are not affected by joint disease. [diki.pl]
Waldenström hyperglobulinemic purpura Other names Purpura hyperglobulinemica Specialty Dermatology Waldenström hyperglobulinemic purpura is a skin condition that presents with episodic showers of petechiae (small red or purple spots) occurring on all [en.wikipedia.org]
Rumpel-Leede sign: distal shower of petechiae that occurs immediately after the release of pressure from a tourniquet release. Associated with capillary fragility. [slideplayer.com]
Discussion HGPW, first described in 1943, involves a combination of recurrent lower-extremity petechiae/purpura, elevated ESR, and polyclonal gammopathy [6]. [hss.edu]
Thus, it is common for the first signs of petechiae due to thrombocytopenia to appear at the ankles. [medtextfree.wordpress.com]
Neurologic
- Irritability
Atopic dermatitis, psoriasis, psoriasiform-id reaction, fungal infections, and irritant contact dermatitis can be differentiated on clinical grounds. [pedsinreview.aappublications.org]
Correlation of tear fluorescein clearance and Schirmer test scores with ocular irritation symptoms. Ophthalmology 1999; 106:803. 15. van Bijsterveld, OP. Diagnostic tests in the sicca syndrome. Arch Ophthalmol 1969; 82:10. 16. [dry.org]
Features such as isolated leucopoenia, urticaria, trigeminal neuropathy, irritable bowel syndrome and minor extra glandular disease features such as minor musculoskeletal features including arthralgia, chronic non-deforming non-erosive polyarthritis, [openrheumatologyjournal.com]
Purpuric eruptions can result from allergic or irritant contact dermatitis to benzoyl peroxide,89 clothing, cobalt, rubber, woolen garments, elastic dyes, Balsam of Peru,90 local anesthetic (EMLA) cream,91,92 or detergent whiteners, and these may simulate [medtextfree.wordpress.com]
Workup
(See Pathophysiology, Etiology, and Workup.) [emedicine.medscape.com]
A thorough workup is needed to diagnose the underlying condition. We report two patients presenting with LCV who were found to have multiple myeloma on investigation. [ijdvl.com]
^ a b Waldenstrom Macroglobulinemia~workup at eMedicine ^ a b National Cancer Institute. Waldenström Macroglobulinemia: Questions and Answers Archived 2013-01-28 at the Wayback Machine. [en.wikipedia.org]
Serum
- Hyperglobulinemia
Bing J: Further investigations of hyperglobulinemia, occurrence and degree of hyperglobulinemia in various diseases. Ratio between hyperglobulinemia, hyperproteinemia and hypoalbuminemia (Formol gel-reaction), Acta med. Scandinav. 103: 547, 1940. [acpjournals.org]
Wal·den·ström mac·ro·glob·u·lin·e·mi·a ( vahld'ĕn-strum ), macroglobulinemia occurring in old people, characterized by proliferation of cells resembling lymphocytes or plasma cells in the bone marrow, anemia, increased sedimentation rate, and hyperglobulinemia [medical-dictionary.thefreedictionary.com]
Humerfelt, S.: Purpura with Hyperglobulinemia, Nord. med. 34:1101, 1947. 8. Kay, H. E. M., and Robertson, K. M.: Benign Hyperglobulinaemic Purpura, J. Path. & Bact. 70:543, 1955. 9. Lindeboom, G. [deepdyve.com]
Purpura hyperglobulinemia. Arch Dermatol. 1966 Nov;94(5):536–541. [PubMed] [Google Scholar] STRAUSS WG. Purpura hyperglobulinemia of Waldenström; report of a case and review of the literature. N Engl J Med. 1959 Apr 23;260(17):857–860. [ncbi.nlm.nih.gov]
- Hyperglobulinemia
Bing J: Further investigations of hyperglobulinemia, occurrence and degree of hyperglobulinemia in various diseases. Ratio between hyperglobulinemia, hyperproteinemia and hypoalbuminemia (Formol gel-reaction), Acta med. Scandinav. 103: 547, 1940. [acpjournals.org]
Wal·den·ström mac·ro·glob·u·lin·e·mi·a ( vahld'ĕn-strum ), macroglobulinemia occurring in old people, characterized by proliferation of cells resembling lymphocytes or plasma cells in the bone marrow, anemia, increased sedimentation rate, and hyperglobulinemia [medical-dictionary.thefreedictionary.com]
Humerfelt, S.: Purpura with Hyperglobulinemia, Nord. med. 34:1101, 1947. 8. Kay, H. E. M., and Robertson, K. M.: Benign Hyperglobulinaemic Purpura, J. Path. & Bact. 70:543, 1955. 9. Lindeboom, G. [deepdyve.com]
Purpura hyperglobulinemia. Arch Dermatol. 1966 Nov;94(5):536–541. [PubMed] [Google Scholar] STRAUSS WG. Purpura hyperglobulinemia of Waldenström; report of a case and review of the literature. N Engl J Med. 1959 Apr 23;260(17):857–860. [ncbi.nlm.nih.gov]
- Microcytic Anemia
The heterozygous form of the disease (thalassaemia minor) is mild and usually asymptomatic, the only manifestation being hypochromic microcytic anemia. [ijdr.in]
Treatment
Broaden your knowledge with updated information on serological diagnosis of pemphigus, TNF-I for hidradenitis suppurativa, the use of immunosuppressives for atopic dermatitis, excimer laser for the treatment of vitiligo and much more. [books.google.com]
Herein we describe a case of a severe PSS patient refractory to conventional treatment as well as, with a notable response to bortezomib, a proteasome inhibitor commonly used for the treatment of multiple myeloma. [ard.bmj.com]
1 Mycophenolate mofetil treatment of a patient with hyperglobulinemic purpura. ( 15455483 ) 2004 2 Therapeutic effect of ethamsylate in a case of hyperglobulinemic purpura associated with Sjogren's syndrome. ( 8833011 ) Casas H.A. 1996 3 Benign hyperglobulinemic [malacards.org]
Prognosis
The exocrine manifestations, diagnosis, treatment, and prognosis of both sicca and extraglandular manifestations are discussed separately. [uptodate.com]
Pathophysiology is emphasized throughout, providing a sound basis for discussions of the diagnosis, treatment, and prognosis that follow. [books.google.de]
E-selectin: High concentrations in patient sera correlate with low incidence of metastasis and, consequently, correlate with good prognosis. Expression of elevated E-selectin facilitates metastasis. [clinicaladvisor.com]
The prognosis of SBN is severe; however, some authors have reported prolonged complete remission [ 8, 10 – 12 ]. 4. Conclusion BNS is a rare and potentially treatable complication of WM. [hindawi.com]
Etiology
English : BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology [decs.bvs.br]
Etiology: This systemic vasculitis is of uncertain etiology. [rheumaknowledgy.com]
Williams Dermatology Service, Veterans Administration Medical Center, and Departments of Dermatology and Pediatrics, University of California School of Medicine, San Francisco Abstract Seborrheic dermatitis is a common skin condition of infancy of unknown etiology [pedsinreview.aappublications.org]
Etiology Cryoglobulinemias are associated with a variety of illnesses (listed below), three most common categories being: infections, autoimmune disorders, and malignancies. [clinicaladvisor.com]
McKee's Pathology of the Skin is the most complete, in-depth resource on dermatopathology, covering etiology, pathogenesis, disease mechanisms, and recent genetic, molecular, and basic science data. Drs. J. [books.google.de]
Epidemiology
Allowable Qualifiers English : BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]
Epidemiology : 2% of all hematologic malignancies. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. [ufrgs.br]
Epidemiology Waldenström macroglobulinemia is a relatively rare condition, with the 1500 cases diagnosed per year in the United States accounting for approximately 2% of hematologic malignancies. [emedicine.medscape.com]
We review the literature on BNS and discuss its epidemiology, presentation, diagnosis, treatment, and prognosis. 2. Case Presentation A 57-year-old man, heavy smoker, was treated since November 2003 for a WM. [hindawi.com]
Pathophysiology
Pathophysiology is emphasized throughout, providing a sound basis for discussions of the diagnosis, treatment, and prognosis that follow. [books.google.de]
[…] caused by organisms growing in vessel and vessel walls (usually immunocompromised host) o Vessel-invasive fungus ( Mucor, Aspergillus, Cephalosporium, Rhizopus, etc.) o Ecthyma gangrenosum ( Pseudomonas ) o Disseminated strongyloidiasis · Uncertain pathophysiology [dermaamin.com]
Pathophysiology of circulating immune complexes. Arthritis Rheum, 1982, 125, 783–787. Google Scholar 13. Clark, R.A., Abraham, G.N., Kyle, R., Vaughan, J.H. Gammaglobulin complexes in rheumatoid arthritis and hypergammaglobulinemic purpura. [link.springer.com]
Further such reports will help augment our understanding on the pathophysiology of IgA-VN in WD. Author Details Authors Ratna Acharya1 Xu Zeng2 William L. [dustri.com]
Prevention
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]
[…] diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PS parasitology PA pathology PP physiopathology PC prevention [decs.bvs.br]
0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis [medicbind.com]
Overview Hypogammaglobulinemia is a problem with the immune system that prevents it from making enough antibodies called immunoglobulins. [healthline.com]