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]

The clinical presentation includes chronic diarrhea with evidence of malabsorption. [medigoo.com]

[…] function is the typical clinical presentation of respiratory variant of alpha HCD. [atlasgeneticsoncology.org]

Diagnosis usually requires bone marrow examination; vacuolated plasma cells are present in two thirds of patients and, when present, are virtually pathognomonic. Death can occur in a few months or in many years. [merckmanuals.com]

• Massive Splenomegaly

  Clinica Chimica Acta, 413, 1696-1699. http://dx.doi.org/10.1016/j.cca.2012.04.020 [ 3 ] Zhang, L., et al. (2013) Unusual Concurrence of T-Cell Large Granular Lymphocytic Leukemia with Franklin Disease Manifested with Massive Splenomegaly. [scirp.org]

• Turkish

  We report on a 28 year old Turkish woman, who was admitted to our hospital with the symptoms of malabsorption and protein-loosing enteropathy. [ncbi.nlm.nih.gov]

  Regression of immunoproliferative small intestinal disease after eradication of Helicobacter pylori. ( 8988128 ) Fischbach W....MA1ller-Hermelink. 1997 40 Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease: a Turkish [malacards.org]

  Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease: a Turkish experience. Cancer. 1997;80:8–14. [tropicalgastro.com]

  : Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease: a Turkish experience. Cancer 1997, 80:8–14. PubMed CrossRef Google Scholar 32. [link.springer.com]

  (Akbulut et al., 1997) Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease: a Turkish experience Akbulut H, Soykan I, Yakaryilmaz F, Icii F, Aksoy F, Haznedaroglu S, Yildirim S Cancer 1997 Jul 1;80(1):8- [atlasgeneticsoncology.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

  (Halphen et al., 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 [atlasgeneticsoncology.org]

• Night Sweats

  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]

• Pulmonary Infiltrate

  A lymphomatous variant with predominant involvement of lymph nodes, spleen, and liver; as well as a respiratory variant with diffuse pulmonary infiltrates and restrictive pattern of respiratory function, have been reported. [atlasgeneticsoncology.org]

• Hyperglobulinemia

  : A case of mu heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test. Ann Hematol 1998, 77:231–234. PubMed CrossRef Google Scholar 21. [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]

  "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). [ipfs.io]

• 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]

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]

Treatment consists of antibiotics and improved nutrition and hygiene. Surgery is occasionally required for patients with bulky masses at risk for bowel perforation. [link.springer.com]

Initial treatment consists of eradication of any concurrent infection (eg, parasites, viruses, Helicobacter pylori, Campylobacter jejuni) with appropriate antibiotics. [medigoo.com]

Treatment depends on the patient’s condition but may consist of alkylating agents plus corticosteroids or may be similar to treatment of the lymphoproliferative disorder that it most closely resembles. [merckmanuals.com]

Treatment usually includes chemotherapy drugs and corticosteroids. Length of survival and response to treatment vary widely. NOTE: This is the Consumer Version. [msdmanuals.com]

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]

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

Prognosis This will depend on the underlying cause. Prognosis is often poor. However, in patients with benign monoclonal gammopathy, patients are asymptomatic and there is no evidence of impairment of antibody response or bone marrow function. [patient.info]

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]

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]

[…] 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]

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]

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. [en.wikipedia.org]