Being rare and probably underdiagnosed diseases the aim of this report is to show an additional case of gamma heavy chain disease in a 48 year old female patient with rheumatoid arthritis focusing on the laboratory presentation. [ncbi.nlm.nih.gov]

In this case serum and urine electrophoresis, and immunofixation studies which are simple and affordable tests facilitated the hematologic workup and follow up. [ncbi.nlm.nih.gov]

CLINICAL PRESENTATION: Patient 1 was a 70-year-old woman, with lymphoplasmacytic lymphoma, refractory to chlorambucil-rituximab treatment. [ncbi.nlm.nih.gov]

Its prevalence and prognosis are unknown. [ncbi.nlm.nih.gov]

Dx: demonstration of anomalous serum M component (often 5. heavy-chain diseases  -HCD Clinical course and prognosis: variable, but mostly rapid downhill course some survives 5 years with chemotherapy death from infection www.freelivedoctor.com 6. most [slideshare.net]

The prognosis is not so great, with survival on the order of months to a few years. Mu chain disease is very rare, and the prognosis is more variable, on the order of months to many years. [pathologystudent.com]

Prognosis The clinical course is variable, depending on subtype. Outcome is very good in patients without lymphoma, in whom prognosis is related to underlying autoimmune disease. [atlasgeneticsoncology.org]

[…] chronic intestinal infection as etiologic stimulation) heavy-chain diseases  -HCD www.freelivedoctor.com 7. defect in assembly of light and heavy chains may seen with CLL/SLL Clinical feature: Bence Jones proteinuria (kappa light chain in urine) increased [slideshare.net]

[…] blood, skin, subcutaneous tissue Mu: spleen, liver, bone marrow, blood, usually not lymphadenopathy Pathophysiology All: deletions in heavy chain gene result in defective heavy chain protein that cannot bind to light chain to form complete Ig molecule Etiology [pathologyoutlines.com]

Etiology Deletions, insertions or point mutations in the constant 1 (CH1) domain of the IgH are acquired during the process of somatic hypermutation. [atlasgeneticsoncology.org]

Etiology, Epidemiology and Pathophysiology Etiology The cause or causes of myeloma are unknown, but there is some evidence to support a number of theories of its origin, including viral, genetic, and exposure to toxic chemicals, the most notable being [cancer.ucsf.edu]

Nevertheless, the underlying etiologic agents “remain[ed] ill defined,” and they concluded that a variety of infectious agents and perhaps other stimuli might play a role. [nap.edu]

Kyle Springer Science & Business Media, ٣١‏/٠٧‏/٢٠١٢ - 1431 من الصفحات Neoplastic Diseases of the Blood integrates the history, epidemiology, pathology, pathophysiology, and therapeutics of modern neoplastic hematopathology. [books.google.com]

October 01, 2015) Signs and Symptoms Fever (alpha & gamma) Hypocalcemia (alpha) Malabsorption (alpha) Wasting (alpha) Weakness (gamma) Weight loss (gamma) Diagnostic Exams Immunophenotyping Peripheral blood smear Progression and Transformation None Epidemiology [seer.cancer.gov]

[…] autoimmune disease, may be polymorphic Mu may resemble chronic lymphocytic leukemia (CLL) with mix of lymphocytes and characteristic vacuolated plasma cells Terminology Alpha heavy chain is also known as immunoproliferative small intestinal disease (IPSID) Epidemiology [pathologyoutlines.com]

Plasma cell disorders can be considered as a spectrum of conditions ranging from monoclonal gammopathy of undetermined significance (MGUS), through asymptomatic, to symptomatic myeloma. [ 1 ] Epidemiology The incidence of a paraprotein is 3.2% in people [patient.info]

2003) Epidemiology Predominantly women with median age at diagnosis 51-68 years. Clinics The clinical presentation of gamma HCD varies according to disease subtype. [atlasgeneticsoncology.org]

Kyle Springer Science & Business Media, ٣١‏/٠٧‏/٢٠١٢ - 1431 من الصفحات Neoplastic Diseases of the Blood integrates the history, epidemiology, pathology, pathophysiology, and therapeutics of modern neoplastic hematopathology. [books.google.com]

Pathophysiology of ECM Accumulation A striking feature of MIDD is the dramatic accumulation of ECM, whose pathogenesis is beginning to be understood. [cjasn.asnjournals.org]

[…] usually not bone marrow or other organs Gamma: lymph nodes or extranodal, Waldeyer ring, gastrointestinal tract, bone marrow, liver, spleen, peripheral blood, skin, subcutaneous tissue Mu: spleen, liver, bone marrow, blood, usually not lymphadenopathy Pathophysiology [pathologyoutlines.com]

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Monoclonal proteins are markers of plasma cell proliferative disorders. [mayomedicallaboratories.com]

Pathophysiology Malignant plasma cells develop from an immune cell called a B lymphocyte. [cancer.ucsf.edu]

In mu HCD, the B cells produce light chains, but they can’t be attached to the mu heavy chains because there are abnormalities in the mu chains that prevent the assembling of complete Ig molecules. [pathologystudent.com]

Infiltration of the intestinal tract wall by cancerous plasma cells often prevents proper absorption of nutrients from food ( malabsorption ), resulting in severe diarrhea and weight loss. A rare form affects the respiratory tract. [msdmanuals.com]

"Immunoglobulin aggregation leading to Russell body formation is prevented by the antibody light chain". Blood. 115 (2): 282–8. doi : 10.1182/blood-2009-07-234864. PMID 19822901. [en.wikipedia.org]

: Immunoproliferative small intestinal disease: portrait of a potentially preventable cancer from the Third World. Am J Med 1990, 89:483–490. PubMed CrossRef Google Scholar 36. [link.springer.com]