Of 10 patients with low-grade disease at presentation, two died--one at 76 months, the other after transforming to high-grade lymphoma at 73 months. Eight patients with low-grade disease are alive, an average of 67 months after presentation. [ncbi.nlm.nih.gov]

• Massive Splenomegaly

  et al. (2013) Unusual Concurrence of T-Cell Large Granular Lymphocytic Leukemia with Franklin Disease Manifested with Massive Splenomegaly. [scirp.org]

• Intermittent Fever

  Patients with Franklin disease usually have a history of progressive weakness, fatigue, intermittent fever, night sweats and weight loss and may present with lymphadenopathy (62%), splenomegaly (52%) or hepatomegaly (37%). [en.wikipedia.org]

• Hypoxemia

  1986) Patients with lymphomatous variant of alpha HCD present with generalized lymphadenoapthies and hepatosplenomegaly, while dyspnea and hypoxemia in the context of diffuse pulmonary infiltrates and restrictive pattern of respiratory function is [atlasgeneticsoncology.org]

• Cutaneous Manifestation

  et al. (1990) Cutaneous Manifestations Associated with Gamma Heavy Chain Disease. Report of an Unusual Case and Review of Literature. [scirp.org]

  : Cutaneous manifestations associated with gamma heavy chain disease: report of an unusual case and review of literature. J Am Acad Dermatol 1990, 23:988–991. PubMed CrossRef Google Scholar 12. [link.springer.com]

• Penicillium

  "Penicillium pneumonia in a patient with newly diagnosed Franklin disease". Am J Med Sci. 344 (1): 69–71. doi : 10.1097/MAJ.0b013e31824a8927. PMID 22543591. Wahner-Roedler DL, Witzig TE, Loehrer LL, Kyle RA (2003). [en.wikipedia.org]

• Colonic Ulcer

  Ultrastructural and immunohistochemical studies of the small intestine are described in a Japanese patient with alpha heavy chain disease who had a history of colonic ulcers. [ncbi.nlm.nih.gov]

Treatment with cyclophosphamide and corticosteroids produced an improvement in the patient's condition and he survived for 18 months after the onset of symptoms. [ncbi.nlm.nih.gov]

Significant updates on bariatric surgery, Barrett's esophagus, endoscopic ultrasound, endosonography, treatment of liver disease, and much more keep you current on the latest advances. [books.google.com]

Overall prognosis is poor but the recent use of doxorubicin-based chemotherapy offers some hope for the future. [ncbi.nlm.nih.gov]

The disease course of alpha-HCD is variable and long-term prognosis of the disease is imprecise. Without antibiotics and chemotherapy the disease progresses rapidly and prognosis is poor. [medigoo.com]

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 is extremely variable. Low-grade disease may respond to chemotherapy. 2. Mu heavy chain disease - resembles CLL (9823/3) because of its small, round lymphocyte s but it is a distinctly different entity. This is the rarest HCD. [seer.cancer.gov]

[…] lymphoma with IgA deposition (IPSID) Clinical Usually associated with MGUS or overt plasma cell myeloma 85% have monoclonal serum immunoglobulin (M component) Usually kappa Symptomatic deposition frequently occurs in Kidneys Liver Heart Joints Poor prognosis [surgpathcriteria.stanford.edu]

Although an infectious etiologic agent is suspected, none have been definitively identified to date. [atlasgeneticsoncology.org]

[…] 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 AND PATHOGENESIS The etiology of heavy-chain disease is not known. Some of the factors responsible for this disease may be similar to those involved in the etiology and pathogenesis of plasma cell myeloma or chronic lymphocytic leukemia. [medtextfree.wordpress.com]

Etiological spectrum of sporadic malabsorption syndrome in northern Indian adults at a tertiary hospital. Indian J Gastroenterol. 2004;23:94–8. Ghoshal UC, Mehrotra M, Kumar S, Ghoshal U, Krishnani N, Misra A, et al. [tropicalgastro.com]

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]

We discuss the clinical presentation; epidemiology; laboratory, radiologic, and pathologic features; and treatment options for each of the heavy chain diseases, emphasising the importance of an accurate pathologic diagnosis and correct interpretation [ncbi.nlm.nih.gov]

Kyle Springer Science & Business Media, ٢٢‏/٠٩‏/٢٠١٢ - 1431 من الصفحات Neoplastic Diseases of the Blood integrates the history, epidemiology, pathology, pathophysiology, and therapeutics of modern neoplastic hematopathology. [books.google.com]

Synonym: Mediterranean abdominal lymphoma Epidemiologic features: (1) It usually occurs in patients from the Middle East and areas around the Mediterranean Sea (Israel, Egypt, North Africa, Saudi Arabia). (2) Patients usually have a low socioeconomic [meducator3.net]

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]

Kyle Springer Science & Business Media, ٢٢‏/٠٩‏/٢٠١٢ - 1431 من الصفحات Neoplastic Diseases of the Blood integrates the history, epidemiology, pathology, pathophysiology, and therapeutics of modern neoplastic hematopathology. [books.google.com]

Pathophysiology The pathogenesis of mu-HCD is not completely understood. [emedicine.medscape.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]

Our understanding of such modulating forces, hopefully, will someday allow us to modify the microenvironment in a favorable way and attain the goal of cancer prevention. [books.google.com]

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]