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]

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]

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

• Pain

  Weight loss, diarrhea, and abdominal pain were the most common complaints and clubbing the most frequent physical findings. [ncbi.nlm.nih.gov]

  Patients present with symptoms of malabsorption.Diarrhea, weight loss and abdominal pain are common. Parasitic infections are often present. Infiltration of the jejunal mucosa with plasmacytoid cells is the most frequent pathologic feature. [medigoo.com]

  Often nonspecific presentation with fever, malaise, and bone pain. Clinical indications for screening for M-protein: [ 1 ] Malaise and fatigue. Bone disease (persistent back pain, osteopenia or lytic lesions). Impaired renal function. [patient.info]

• Weight Loss

  A 57-year-old Japanese man was admitted to our hospital with diarrhea, weight loss and malabsorption. Due to a high serum IgA level, we suspected α-heavy chain disease (α-HCD). [ncbi.nlm.nih.gov]

  Patients present with symptoms of malabsorption.Diarrhea, weight loss and abdominal pain are common. Parasitic infections are often present. Infiltration of the jejunal mucosa with plasmacytoid cells is the most frequent pathologic feature. [medigoo.com]

• Hodgkin Lymphoma

  Página 768 - The Non-Hodgkin's Lymphoma Pathologic Classification Project. National Cancer Institute sponsored study of classifications of Non-Hodgkin's lymphomas. Summary and description of a Working Formulation for clinical usage. ‎ [books.google.es]

  The Non-Hodgkin’s lymphoma pathologic classification project: National Cancer Institute sponsored study of classifications of Non-Hodgkin’s lymphomas. Cancer, 1982;49:2 112-2135. 40. Jones, SE., Fuks, Z., Bull, M. et at. Non-Hodgkin’s lymphomas IV. [jpma.org.pk]

  古典的ホジキンリンパ腫　Classical Hodgkin lymphoma: CHL (田丸　淳一) 517 3. 結節硬化型古典的ホジキンリンパ腫　Nodular sclerosis classical Hodgkin lymphoma: NSCHL (浅野　直子) 521 4. 混合細胞型古典的ホジキンリンパ腫　Mixed cellularity classical Hodgkin lymphoma: MCCHL (浅野　直子) 523 5. [rnavi.ndl.go.jp]

  If there is no response to antibiotics or if aggressive non-Hodgkin’s lymphoma (NHL) is diagnosed, the patient should be treated with chemotherapy. [link.springer.com]

• Swelling

  Fine needle aspiration from the swelling showed plasmacytoma. A tissue biopsy confirmed the diagnosis of plasmacytoma. Bone marrow examination showed 9% plasma cells, the majority being immature forms. Hemogram revealed mild anemia. [ijpmonline.org]

  Other changes that may occur include hair growth on the face, limbs and chest, swelling (edema) of the legs and feet, and whitening of the nails. [cancer.ca]

  The waxing and waning lymphadenopathy and palatal and uvular swelling on the basis of involvement of Waldeyer’s ring, described in the early cases of the disease, are not as frequent as originally believed. [medtextfree.wordpress.com]

  Prednisone and dexamethasone can be given with chemotherapy agents or alone for people who cannot tolerate chemotherapy drugs or need them to help to lower calcium levels or to reduce swelling around nerves being pressed upon by masses of plasma cells [emedicinehealth.com]

  Symptoms include pain, swelling, loose teeth, exposed bone, drainage, and infection. This occurs in somewhere between 1 % and 5% of people who have been on the drugs for many years. [cancer.ucsf.edu]

• Congestive Heart Failure

  Cardiac involvement may be responsible for cardiomegaly and severe heart failure. Arrhythmias, conduction disturbances, and congestive heart failure are seen. [cjasn.asnjournals.org]

  heart failure caused by an increased blood volume meant to dilute the blood). [emedicinehealth.com]

  Manifestations of this disease include thick tongue, hoarse voice, congestive heart failure, dizziness upon standing, tingling or pain in the toes and fingers, and diarrhea. [cancer.ucsf.edu]

• Aspiration

  Diagnosis and typing of systemic amyloidosis: The role of abdominal fat pad fine needle aspiration biopsy. Cytojournal. 2010 Jan 15;6:24. [surgpathcriteria.stanford.edu]

  Bone marrow aspiration and trephine biopsy. Management Management will depend on the underlying cause. Prognosis This will depend on the underlying cause. Prognosis is often poor. [patient.info]

  Monoclonal-plasma-cell proliferation was not detected by skeletal x-ray survey, bone marrow aspiration and biopsy, thyroidectomy, or cytoplasmic immunofluorescence studies of bone marrow and thyroid cells with the use of antibodies to immunoglobulin heavy [nejm.org]

  Bone marrow biopsy or aspiration In a bone marrow biopsy, your doctor will remove a small piece of bone that contains marrow. For a bone marrow aspiration, your doctor will remove a small amount of liquid bone marrow, which contains marrow cells. [themmrf.org]

  Fine needle aspiration from the swelling showed plasmacytoma. A tissue biopsy confirmed the diagnosis of plasmacytoma. Bone marrow examination showed 9% plasma cells, the majority being immature forms. Hemogram revealed mild anemia. [ijpmonline.org]

• Diarrhea

  A 20-yr-old black male was admitted with a 5-month history of profound weight loss and diarrhea. Appetite and dietary intake had been remarkably well preserved up until the week before admission. [ncbi.nlm.nih.gov]

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

• Abdominal Pain

  Weight loss, diarrhea, and abdominal pain were the most common complaints and clubbing the most frequent physical findings. [ncbi.nlm.nih.gov]

  Patients present with symptoms of malabsorption.Diarrhea, weight loss and abdominal pain are common. Parasitic infections are often present. Infiltration of the jejunal mucosa with plasmacytoid cells is the most frequent pathologic feature. [medigoo.com]

  Clinical features: (1) The disease usually affects young adults (20's or 30's). (2) The patient may present with: (2a) diarrhea, steatorrhea, malabsorption (2b) fever (2c) abdominal pain (2d) wasting (2e) hypocalcemia (3) The patient may have a history [meducator3.net]

• Nausea

  (Doe et al., 1972) Patients can report nausea and emesis, while alopecia, amenorrhea, and growth retardation are present in severe cases. [atlasgeneticsoncology.org]

  Depending on how and where the radiation is administered, it may cause certain side effects such as fatigue, loss of appetite, nausea, diarrhea, urgency of urination, and skin problems. [emedicinehealth.com]

  Abnormally high levels of calcium may result in nausea, lack of appetite, fatigue, abdominal pain, muscle pain and weakness, excessive thirst, and/or confusion. [rarediseases.org]

• Vomiting

  Hypercalcemia and kidney damage: Common symptoms include fatigue, nausea, vomiting, loss of appetite, muscle weakness, constipation, decreased urine production, dehydration and increased thirst, restlessness, difficulty in thinking or concentrating, and [emedicinehealth.com]

• Heart Failure

  […] with cardiac failure from ischemic or valvular heart disease and from 13 normal subjects who were cardiac antibody-negative. [circ.ahajournals.org]

  Rapidly progressive renal failure or the nephrotic syndrome is the most common manifestation, but heart failure, arrhythmia, liver involvement (peliosis), and neurologic disorders may also be present. [nejm.org]

  Cardiac involvement may be responsible for cardiomegaly and severe heart failure. Arrhythmias, conduction disturbances, and congestive heart failure are seen. [cjasn.asnjournals.org]

  Amyloidosis: The symptoms are related to malfunction or failure of organs or structures infiltrated by amyloid deposits. These symptoms include problems such as heart failure, liver failure, kidney failure, and blood vessel damage. [emedicinehealth.com]

  Manifestations of this disease include thick tongue, hoarse voice, congestive heart failure, dizziness upon standing, tingling or pain in the toes and fingers, and diarrhea. [cancer.ucsf.edu]

• Hypertension

  Delivery Models Health Care Economics, Insurance, Payment Health Care Policy Health Care Quality Health Care Reform Health Care Safety Health Care Workforce Health Disparities Health Informatics Health Policy Hematology History of Medicine Humanities Hypertension [jamanetwork.com]

  Methods Patients Patient 1, a 53-year-old woman, was referred because of hypertension, leg edema, exertional dyspnea, and anemia of five months' duration. Her urinary protein excretion was 4.4 g per 24 hours. [nejm.org]

  The prevalence of hypertension is variable, but it must be interpreted according to associated medical history. [cjasn.asnjournals.org]

  Megaloblastic anemia Protein-losing enteropathies Severe liver disease Wilson’s disease Increased beta 1 or beta 2 globulins Biliary cirrhosis Carcinoma (sometimes) Cushing’s disease Diabetes mellitus (some cases) Hypothyroidism Iron deficiency anemia Malignant hypertension [aafp.org]

• Kidney Failure

  Amyloidosis: The symptoms are related to malfunction or failure of organs or structures infiltrated by amyloid deposits. These symptoms include problems such as heart failure, liver failure, kidney failure, and blood vessel damage. [emedicinehealth.com]

  Renal Failure Patients with myeloma often present with renal failure and the cause of the kidney injury is often multifactorial. Hypercalcemia and hyperuricemia can occur as a result of myeloma and can contribute to renal failure. [cancer.ucsf.edu]

• Ataxia

  The Vysis CLL FISH Probe Kit uses FISH DNA probe technology to determine deletion status of probe targets for locus-specific identifier (LSI) TP53 (containing tumor protein p53 gene, located on chromosome 17p), LSI ATM (containing ataxia telangiectasia [molecular.abbott]

• Helicobacter Pylori

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

  pylori. ( 20300878 ) Dutta U....Singh K. 2010 14 Immunoproliferative small intestinal disease versus colonic monoblastic sarcoma in a 2-year-old boy. ( 19942975 ) Miron I....MoisA8 S.M. 2009 15 Immunoproliferative small intestinal disease with protein [malacards.org]

  Helicobacter pylori infection and gastric lymphoma. N Engl J Med 1994; 330 : 1267–1271. 103. Wotherspoon, AC, Doglioni, C, Diss, TC, et al. [nature.com]

  Regression of immunoproliferative small intestinal disease after eradication of Helicobacter pylori. Lancet. 1997;349:31–2. Lecuit M, Abachin E, Martin A, Poyart C, Pochart P, Suarez F, et al. [tropicalgastro.com]

  Interestingly, many of these patients may have concomitant parasitic infestation or chronic infection with bacteria, such as Helicobacter pylori. [medtextfree.wordpress.com]

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 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]

Treatments Treatment for amyloidosis is similar to treatment for multiple myeloma. It targets the abnormal plasma cells. [cancer.ca]

This form of treatment is sometimes referred to as "consolidation therapy." If this treatment does not induce a complete remission, the person will probably be given a different treatment regimen. [emedicinehealth.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]

It is very important for you to undergo all of the appropriate tests, as the results will help your doctor better determine treatment options and prognosis. [themmrf.org]

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. [ipfs.io]