Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 60-year-old man was admitted with general fatigue and jaundice of one year's duration in February, 1981. The hemoglobin (Hb) was 11.4 g/dl and reticulocytes were 1.7%. A diagnosis of chronic cold agglutinin disease (CCAD) was made from the presence of cold agglutinin (CA) 1:2,048, increased serum IgM 267 mg/dl and indirect bilirubin 1.4 mg/dl. His Hb was approximately 11 g/dl in summer and 9 g/dl in winter for the subsequent ten years without therapy. In July, 1990, he was readmitted because of exacerbation of anemia and hepatosplenomegaly. The Hb was 4.6 g/dl, indirect bilirubin 3. 1 mg/dl, CA titer 1:232,144 and reticulocytes were 20%. Serum IgM was 1,065 mg/dl, and immunoelectrophoresis showed IgM-kappa M-protein. Peripheral blood lymphoid cells expressed surface membrane immunoglobulin (SmIg) M and kappa. The bone marrow showed an increased number of lymphoid cells which also expressed SmIg M and kappa. These findings were compatible with those of the features of primary macroglobulinemia (PMG). The M-2 protocol resulted in decrease in serum IgM and CA, but he died of heart failure in February, 1991. The relationship between CCAD and PMG in relation to the pathogenesis was discussed.
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PMID:[Chronic cold agglutinin disease terminating in primary macroglobulinemia after a 10 year history]. 146 90

A male aged 58 was admitted to our hospital because of proteinuria, hematuria and bilateral pretibial edema. Laboratory tests showed normocytic, normochromic anemia and moderately impaired renal function. Antinuclear antibodies were negative. Neither M-protein nor Bence-Jones protein were detected. Light microscopic study on the biopsied renal specimen indicated a moderate mesangial proliferation accompanying with the deposition of PAS-positive and Congo red-negative materials in the subendothelial area. C3 accumulated segmentally along the capillary walls, which was clarified by immunofluorescence microscopy. Staining for IgG, IgA, IgM and light chains were negative. Electron microscopy demonstrated the deposition of microtubules in the mesangial, subepithelial and subendothelial areas. The diameter of these microtubules ranged from 40 to 80 nm. Such type of the microtubules have been reported to exist in the glomeruli in the patients with systemic diseases such as amyloidosis, systemic lupus erythematosus, cryoglobulinemia and light chain disease. In our patient, however, any clinical or serological findings suggestive of these systemic diseases were not obtained. On the other hand previous report pointed out that microtubules deposited in the glomeruli in the patients with immunotactoid glomerulopathy or other glomerulopathies. Our patient had the clinical features consistent with these glomerulopathies. However, no depositions of immunoglobulins were observed. This case is an atypical glomerulopathy accompanying with the glomerular microtubular deposits.
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PMID:[Glomerulopathy associated with glomerular microtubular deposits: a report of a case]. 148 13

A 51-year-old male was admitted because of eyelid edema and anosmia, which developed in 1985. He showed bilateral cervical lymphadenopathy, with nodes larger than 2 x 2 cm, and remarkable eyelid edema. Anemia, hyperimmunoglobulinemia with hypo-albuminemia (no M-protein) and liver dysfunction were found. Bone marrow, renal function, urine analysis and LDH level were normal. Cervical lymph node biopsy showed interfollicular proliferation of plasma cells without any malignant appearance. On day 3 of oral prednisolone (PD), anosmia abruptly improved from day 7, eyelid swelling, cervical lymphadenopathy and gammopathy subsided gradually. Since PD was tapered off, no relapse has been observed thus far.
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PMID:Successful treatment of idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia. 163 68

Plasmacytoma originating in the cranial bone is a rare disease. A report is presented of a case of plasmacytoma originating in the parieto-occipital region. The patient was a 62-year-old male with palpation of a parieto-occipital mass as chief complaint. He showed no neurological deficit. Ig-G in the serum was 2240 mg/dl, and M-protein (n-type) was demonstrated in the serum. Bence-Jones protein was negative and bone marrow was normal. Skull X-ray showed osteolytic change in the parieto-occipital region. CT scan and MRI demonstrated a markedly enhanced mass extending from the epidural to the subcutaneous space at the parieto-occipital region. Common carotid angiography showed remarkable tumor stain flowing from the occipital artery, the superficial temporal artery, and the middle meningeal artery. Following embolization of the bilateral occipital artery, parieto-occipital horse shoe scalp incision was done. The tumor was elastic soft and bled easily around the margin of destroyed bone. Subtotal removal of the tumor was accomplished. The removed surgical specimen of the tumor was found to be plasmacytoma. The patient's postoperative course was favorable and no neurological deficit was found. Laboratory studies revealed a remarkable reduction of Ig-G in the serum to 1170 mg/dl. Six months after the operation, no signs of recurrence were seen on CT scan. Plasmacytoma originating in the cranial bone is so rare that only 18 cases have been reported to date. In these cases, laboratory studies have shown no evidence of anemia, Bence-Jones protein in urine, and abnormality of the bone marrow which are characteristic of multiple myeloma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Plasma cell tumor of the parieto-occipital bone; a case report]. 176 57

A few cases of multiple myeloma associated with myelofibrosis has been reported. In Japan, such cases have been less reported. We report a case of IgD-myeloma with remarkable marrow fibrosis. A 44-year-old female was admitted to our hospital because of back pain. On admission her peripheral blood revealed anemia and leukopenia. Serum immunoelectrophoresis revealed M-protein of IgD and serum IgD level remarkably increased to 5420 mg/dl. Bone marrow aspiration resulted in dry tap every three times and its biopsy at iliac bone showed remarkable infiltration of myeloma cell and remarkable increase of reticulin fiber with marked decrease of normal hematopoietic cells. Her liver and spleen were not palpable and tear drop cells of erythrocytes were not shown in peripheral blood. We could not find view of myelofibrosis with chronic myeloproliferative disorders. Multiple myeloma associated with myelofibrosis is a interesting syndrome as clinical signs and course. So further investigation must be needed.
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PMID:[IgD-lambda multiple myeloma associated with bone marrow fibrosis]. 190 16

Four cases of plasma cell type Castleman's disease (CD) are described. Two patients had localized forms (one mediastinal and the other mesenteric) and presented systemic manifestations associated with hypergammaglobulinemia and severe anemia. In both cases, the lesions were revealed by computerized tomography scans and cures were obtained by the complete surgical removal of the masses, which led to the rapid disappearance of the systemic manifestations. The other 2 patients had the multicentric form of CD and presented more extensive clinical and biological symptoms. One of these developed severe peripheral neuropathy and endocrine anomalies during the late phase of his disease, which led us to discuss the relationship between multicentric CD and the POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change) syndrome first described in Japan. Three of our patients presented with hypochromic microcytic anemia too severe to be explained by an inflammatory syndrome alone, and was likely due to several mechanisms. The etiology of CD remains unknown. The histological characteristics of angiofollicular lymph node hyperplasia are among the most important criteria for the diagnosis of localized and multicentric forms of CD, which can easily be made on a lymph node biopsy. However, it must be noted that this lesion can also be observed (but only rarely) in HIV (human immunodeficiency virus) - infected patients. The localized form is always considered to be benign, but, to date, there is no formal argument definitively supporting the malignancy of the multicentric one, in spite of its clinical similarity to a lymphoproliferative syndrome.
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PMID:[Castleman's disease (giant lymph node hyperplasia): clinical, biological and developing polymorphism. Apropos of 4 cases]. 216 41

Patients with monoclonal gammopathy of undetermined significance (MGUS) have a monoclonal protein but no evidence of multiple myeloma, macroglobulinemia, amyloidosis, or related disorders. In a long-term follow-up (median, 19 years) of 241 patients with MGUS, 53 (22%) developed multiple myeloma (36 patients), macroglobulinemia (7 patients), primary systemic amyloidosis (7 patients), or malignant lymphoproliferative disease (3 patients). Fifty-seven patients (24%) remained stable and were alive, while 124 patients (51%) died of causes unrelated to the monoclonal gammopathy. Multiple myeloma was diagnosed in the 36 patients 23 to 251 months (median, 9.6 years) after recognition of the M-protein. Smoldering multiple myeloma (SMM) is characterized by a serum M-protein level greater than 30 g/l and greater than 10% plasma cells in the bone marrow but no anemia, renal insufficiency, or lytic lesions. Differentiation of MGUS and SMM from multiple myeloma and macroglobulinemia is difficult at the time of diagnosis of the M-protein, and the patients must be followed up indefinitely.
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PMID:Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. 251 9

Diffuse osteosclerotic myeloma is very rare, and primary plasma cell leukemia with extensive osteosclerosis is even more rare. We describe a 71-year-old man who presented with severe anemia and dense widespread osteosclerosis similar to the X-ray finding of myelosclerosis. His peripheral blood showed 40% plasma cells. Bone marrow examination revealed heavy plasma cell infiltration with marked myelofibrosis and myelosclerosis. Protein electrophoresis and immunoelectrophoresis demonstrated an M-protein of IgG-lambda type. He was treated with cyclophosphamide, vincristine, and prednisolone for 10 months. A complete remission was obtained, with disappearance of M-protein and circulating plasma cells and normalization of complete blood counts, bone marrow picture, and biochemical parameters, as well as complete regression of myelofibrosis and osteosclerotic lesions. Unmaintained complete remission lasted for more than 1 year and he survived for more than 22 months. Our case indicated that one must include in the differential diagnosis of an osteosclerotic lesion the possibility of multiple myeloma, and that combination chemotherapy can induce a complete remission in this disease.
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PMID:Primary plasma cell leukemia with extensive dense osteosclerosis: complete remission following combination chemotherapy. 754 34

A 73-year-old male was admitted to the renal division of our hospital because of hypertension, proteinuria and bilateral pretibial edema. Eight years previously, he was diagnosed as being afflicted with interstitial pneumonia on the basis of a chest X-ray examination. Laboratory tests conducted during the current admission showed normocytic normochromic anemia, renal dysfunction and mild proteinuria. Total IgG was normal, but a high proportion of IgG2 was observed. M-protein in the serum was positive for both IgG lambda and Bence Jones protein (lambda type). A bone marrow biopsy showed the proportion of plasma cells to be 10.6%, but atypical cells were not found. We diagnosed the patient's condition as plasma cell dyscrasia. Light microscopy examination of a renal biopsy specimen showed moderate mesangial proliferation with a deposition of PAS-positive and Congo red negative materials in the mesangial area: nodular gomerulonephritis was seen in some glomeruli. Immunofluorescence revealed IgG and lambda light chains, strong linear staining along the glomerular basement membrane and tubular basement membrane and positivity in the mesangial area. Results of staining for IgA, IgM, fibrinogen and C3 were weakly positive in the mesangium area, while those for C4, Clq and free kappa were negative. Positive staining of IgG2 was seen by immunoperoxidase study, but the tissue was negative for IgG1, IgG3, IgG4. Electron microscopy demonstrated a dense granular deposition in the mesangial, subendothelial and peritubular area and a microfibrillar structure in the mesangial area. The diameter of the microfibrillar structure was 14 nm on the average.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case report of light and heavy chain deposition disease (IgG2 lambda)]. 796 78

From October 1983 until December 1988, 50 patients with asymptomatic multiple myeloma stage I were included in a prospective randomized multi-centre study comparing melphalan-prednisone (MP) therapy started at the time of diagnosis with deferred therapy where MP was started at the time of disease progression. Twenty-five patients were randomized to each group. The median time from diagnosis to start of therapy in the group with deferred therapy was 12 months. The reasons for starting therapy were increasing M-protein in 8 cases, symptomatic bone disease in 9 and anaemia in 5. In 2 cases, disease progression was complicated by vertebral fractures necessitating radiotherapy. Two patients in the group in which MP was started at the time of diagnosis developed acute leukaemia. No differences in response rate, response duration or survival were observed between the treatment groups. We conclude that in asymptomatic myeloma deferral of chemotherapy is feasible in well-informed and well-controlled patients but conveys no advantage in survival. In clinical practice the benefits of treatment deferral are to some extent outweighed by disease progression before start of treatment.
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PMID:Initial versus deferred melphalan-prednisone therapy for asymptomatic multiple myeloma stage I--a randomized study. Myeloma Group of Western Sweden. 844 Mar 64


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