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PubMed1992

[A case of small cell lung cancer with Waldenstrom's macroglobulinemia].

Waldenstrom's Macroglobulinemia is characterized by a proliferation of cells of the reticuloendothelial system associated with a monoclonal increase in serum levels of gamma globulins of immunoglobulin M class. We report a case of lung cancer with Waldenstrom's macroglobulinemia. A 68-year-old man was admitted to our hospital for abnormal mass shadow on chest X-P during chemotherapy for macroglobulinemia. Pathological diagnosis was small cell carcinoma by transbronchial lung biopsy. Right middle and lower lobectomy with mediastinal lymph nodes dissection were performed. Postoperative staging was stage II (t1n1m0). Chemotherapy for the lung cancer and plasmapheresis for hyperviscosity syndrome were carried out, after surgical treatment. This patient has been followed for three years and has no evidences of recurrence of the lung cancer.

Aged↗

PubMed1992

Waldenström macroglobulinemia with an IgM-kappa antiepidermal basement membrane zone antibody.

BACKGROUND: Waldenström macroglobulinemia, a lymphoplasmacytoid cell malignant neoplasm associated with a monoclonal IgM paraprotein, has been associated with a number of cutaneous manifestations. On rare occasions, IgM deposits have been demonstrated in the epidermal basement zone of patients with WM. OBSERVATIONS: We report the case of a patient with Waldenström macroglobulinemia and IgM-kappa paraprotein who had development of an eruption of pruritic papules and demonstrated the following unusual immunopathologic findings: (1) deposits of IgM-kappa in the epidermal basement membrane zone of lesional and nonlesional skin; (2) a circulating IgM-kappa antiepidermal basement membrane zone antibody; and (3) binding of this circulating IgM-kappa antiepidermal basement membrane zone antibody to both sides of 1 mol/L sodium chloride split skin. The cutaneous eruption cleared completely with oral psoralen with long-wave UV radiation in the A range (PUVA) therapy. CONCLUSIONS: We present a patient with Waldenström macroglobulinemia who had a distinctive papular eruption and immunopathologic findings suggesting that his paraprotein has specificity for the epidermal basement membrane zone.

Basement Membrane↗

PubMed2004

Lymphoplasmacytic lymphoma with IgA paraproteinemia mimicking Waldenstrom's Macroglobulinemia.

Central to the diagnosis of Waldenstrom's Macroglobulinemia is the demonstration of bone marrow infiltration by lymphoplasmacytic lymphoma with Ig M monoclonal gammopathy. We describe a patient who presented with a clinical and haematological picture, highly suggestive of Waldenstrom's Macroglobulinemia, but whose serum monoclonal immunoglobulin belonged to Ig A class. Ig A secreting lymphoplasmacytic lymphoma undoubtedly exist but are exceedingly uncommon and their relationship to Waldenstrom's Macroglobulinemia needs to be clarified.

Diagnosis, Differential↗

PubMedDate not supplied

[Waldenström's macroglobulinemia with an uncommon presentation].

Waldenström's macroglobulinemia is a rare disease with an indolent clinical course. The median age of the affected patient is 65 years. Nevertheless, we report a case of Waldenström's macroglobulinemia revealed by a splenomegaly and severe pancytopenia, in a 51-year-old man without previous medical history. According to the recent consensus recommendations for the clinicopathological definition of Waldenström's macroglobulinemia, diagnosis was made through morphological and immunophenotypic data of medullary cells. The reduced survival of the patient is associated with the importance of the cytopenia.

Chromosome Mapping↗

PubMed1989

Bilateral central retinal vein occlusion in Waldenström's macroglobulinemia.

Waldenström's macroglobulinemia is a rare progressive neoplastic plasma cell disorder. Hyperviscosity is a major component in primary macroglobulinemia due to the excessive production of IgM paraprotein, a tumor by-product. The ocular manifestations of hyperviscosity can include congestion of the conjunctival vessels, dilation, segmentation and tortuosity of retinal vessels, retinal hemorrhages and serous retinal detachment. This case of bilateral central retinal vein occlusion represents an uncommon complication of Waldenström's macroglobulinemia.

Fundus Oculi↗

PubMed1986

Mid-peripheral hemorrhages secondary to Waldenström's macroglobulinemia.

Waldenström's macroglobulinemia is a progressive neoplastic syndrome of the reticuloendothelial system and is characterized by the presence of monoclonal IgM paraproteins. The ocular manifestations of macroglobulinemia can include mid-peripheral hemorrhages, sludging of blood in the conjunctival vessels, blurred disc margins, exudates in the fundus and serous retinal detachment. Waldenström's macroglobulinemia and its ocular complications are herein presented.

Aged↗

PubMed1985

[Intestinal involvement and exudative enteropathy in Waldenström's macroglobulinemia].

The case of a 60 year old man in whom diarrhea, steatorrhea and protein losing enteropathy developed one year after the diagnosis of Waldenström's macroglobulinemia is reported. Histological study of multilevel peroral biopsies of the small bowel mucosa showed the following features: a) lymphangiectasia, b) intralymphatic and interstitial deposits composed of lipids and immunoglobulin M of the kappa type (as demonstrated by specific histoimmunofluorescence), c) a mild lymphoplasmocytoid infiltrate, corresponding to the location of Waldenström's macroglobulinemia. The patient died six months later, in spite of improvement of his digestive tract functional abnormalities by a low-fat diet. Sixteen cases of small intestinal involvement have been reported during the course of Waldenström's macroglobulinemia, of which only one showed features similar to those found in our case. Lymphangiectasia appear to be due to mesenteric lymph node involvement by the tumoral process, and/or to increased blood viscosity; in turn the lymphangiectasia seem to be responsible for steatorrhea and plasma protein losses, since in our case both lipids and the immunoglobulin M-kappa were shown to cross the villous epithelium by immunohistochemistry.

Humans↗

PubMed1981

Waldenström's macroglobulinemia and myasthenia gravis.

Until now, three patients with IgG and only one with IgM class monoclonal gammopathy (without the classical features of Waldenström's macroglobulinemia) have been reported in subjects with myasthenia gravis. A case of Waldenström's macroglobulinemia which occurred before thymectomy in a myasthenic patient is described in this paper. Both neuromuscular disease and the lymphocyte dyscrasia worsened after operation and before starting steroid treatment. No evidence of circulating immune complexes nor of anti-acetylcholine receptor antibodies belonging to the IgM class was found. The patient's HLA type shared A2 and B15 antigens with an IgG-lambda monoclonal gammopathy previously reported in a myasthenic woman, and his genotype included the Bw15 specificity which has been described to be frequent in Waldenström's macroglobulinemia. It appears that a persistent thymic abnormality, responsible for myasthenia gravis, may be associated with a lymphoreticular neoplasm.

Adult↗

PubMed1995

An autopsy case of B-cell lymphoma associated with macroglobulinemia (IgM-k).

While he was treated for macroglobulinemia showing symptoms, a 65-year-old man developed an enlargement of the left lateral cervical lymph node. Biopsy specimens of the cervical lymph node revealed large-cell non-Hodgkin's lymphoma. The lymphoma cells had rearrangement in the immunoglobulin heavy chain and kappa light chain genes. There was osteolysis involving right metatarsal bones. And, a tumor appeared on the upper hard palate in the terminal stage. Autopsy specimens showed infiltration of lymphoma cells into multiple organs. Production of monoclonal immunoglobulin M was detected only slightly and focally in the tumors. Plasmacytic differentiation as in Waldenström's macroglobulinemia was not seen in any organs except the tumor on the upper hard palate. From these findings, it is speculated that B-cell lymphoma might have developed in the course of macroglobulinemia.

Aged↗

PubMed1995

[von Recklinghausen's disease complicated by macroglobulinemia, malignant schwannoma and pheochromocytoma].

We reported a case of von Recklinghausen's disease complicated by macroglobulinemia, malignant Schwannoma and pheochromocytoma. A 43-year-old male patient who was diagnosed as von Recklinghausen's disease developed abdominal masses at 5 years after the adrenalectomy for pheochromocytoma. Computed tomography showed multiple heterogeneous tumors in abdominal cavity. Histological examination of resected tumor was compatible with malignant Schwannoma. At the same time, peripheral blood and bone marrow smears showed a large number of plasmacytoid lymphocytes. Immunoelectrophoresis revealed M-protein of IgM, kappa type. He was also diagnosed of coexistence with macroglobulinemia. The chemotherapeutic protocols used were not effective against both malignant Schwannoma and macroglobulinemia. The present case is a rare case of von Recklinghausen's disease associated with triple neoplasms.

Abdominal Neoplasms↗

PubMed1996

Ophthalmic tumor formation in Waldenström's macroglobulinemia.

Waldenström's macroglobulinemia is a malignant disease of the reticuloendothelial system; ocular complications reported previously have been concerned chiefly with hyperviscosity manifestations. Ophthalmic tumor formation is rare, although not unheard of, in this particular condition. This report describes two rare instances of Waldenström's macroglobulinemia which involved conjunctival and orbital tumors. One patient developed the tumors after a lengthy clinical course; in the other patient, eyelid and orbital tumors were the first symptoms. Careful examination of the external eye, especially the fornix of the conjunctiva, in patients with Waldenström's macroglobulinemia can identify an initial lesion or invasions to other organs.

Adult↗

PubMed1998

Waldenström macroglobulinemia-induced bullous dermatosis.

BACKGROUND: Waldenström macroglobulinemia is a plasma cell dyscrasia of undetermined cause characterized by the monoclonal proliferation of lymphoplasmacytes in the bone marrow, lymph nodes, and spleen and elevated circulating levels and tissue deposition of monoclonal IgM produced by these aberrant cells. Rarely, cutaneous manifestations of this disease have been reported. OBSERVATIONS: We report the case of a patient with bullous dermatosis induced by Waldenström macroglobulinemia and demonstrate the subepidermal location of the separation and the presence of IgM and kappa light chains by immunoperoxidase, immunofluorescent techniques, and electron microscopy with immunogold staining. Immunoblotting revealed a strong band at the 290-kd area. CONCLUSIONS: The demonstration of the separation in the upper dermis at the site of IgM deposits suggests that these deposits may be an etiologic factor in this rare manifestation.

Adult↗

PubMed1982

Chronic liver disease manifesting as Waldenström's macroglobulinemia.

A 59-year-old woman was initially seen with anemia, purpura, gastrointestinal tract and vaginal bleeding, pronounced hyperglobulinemia, and an increased serum viscosity, all suggestive of Waldenström's macroglobulinemia. Subsequent investigation, however, indicated that these abnormalities were more likely due to chronic active hepatitis with cirrhosis. The striking similarity of this patient's symptoms to those of Waldenström's macroglobulinemia and the pathophysiologic characteristics of polyclonal hypergammaglobulinemia in chronic liver disease are discussed.

Antigens↗

PubMed1980

Amyloid neuropathy and tremor in Waldenström's macroglobulinemia.

We report a case of Waldenström's macroglobulinemia with amyloid neuropathy and a parkinsonian syndrome. We describe the light and electron microscopic findings in a sural nerve biopsy specimen and discuss the possible pathogenic mechanisms in the production of the neuropathy. In contrast to a number of previous cases of neuropathy associated with Waldenström's macroglobulinemia, evidence of immunoglobulin deposition on nerve fibers was not found using immunofluorescent techniques.

Amyloidosis↗

PubMed1998

Waldenström's macroglobulinemia resulting from localized gastric lymphoplasmacytoid lymphoma.

A patient with a high level serum monoclonal IgM lambda paraprotein (3,850 mg/dL) was found to have a mass infiltrating the gastric mucosa. Gastric biopsy with immunohistochemical stains showed a B-cell lymphoplasmacytoid infiltrate expressing IgM lambda, consistent with Waldenström's macroglobulinemia. The patient's response to gastric radiation indicated that the primary source of the macroglobulinemia was the stomach. This is an extremely rare presentation, with only six other reported cases of this entity with gastric involvement.

Female↗

PubMed1977

Acute myelomonocytic leukemia in a patient with macroglobulinemia and malignant lymphoma.

Acute myelomonocytic leukemia occurred in a 69-year-old man with malignant lymphoma and macroglobulinemia. Fluorescent staining demonstrated the macroglobulin in plasma and leukemic cells. Plasma cells seemed to be producing paraproteins, while leukemic cells appeared to be engulfing them as seen with transmission electron microscopy. The sequence of events ruled out the possibility that myelomonocytic leukemia developed following the use of cytotoxic drugs. The case presented in this report is a rare example of malignant lymphoma, macroglobulinemia, and acute myelomonocytic leukemia occurring in the same patient.

Aged↗

PubMed1987

Cutaneous Waldenström's macroglobulinemia. A report of a case successfully treated with radiotherapy.

Waldenström's macroglobulinemia is a lymphoproliferative disorder that only rarely exhibits specific cutaneous findings. The authors present a case of Waldenström's macroglobulinemia in which the patient developed cutaneous lesions while his systemic disease seemed to be under control with chlorambucil therapy. A review of the scant literature regarding the response of cutaneous Waldenström's to treatment suggests that the skin lesions do not respond well to systemic therapy. This case was successfully treated with radiotherapy.

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PubMed1989

Four brothers with Waldenstrom's macroglobulinemia.

An exceptional family including four brothers with Waldenstrom's macroglobulinemia was studied. The four patients had different light chain IgM monoclonal components: two of the kappa type and two of the lambda type. Anti-idiotypic rabbit antisera, prepared for each monoclonal component, revealed no cross-reactivity. The four brothers did not share a common HLA A B DR haplotype and a genetic linkage to the HLA complex cannot be ascertained. Five of the 12 relatives had high serum immunoglobulin concentration (four IgG, three IgA, and two IgM) without monoclonal components. Two relatives showed auto-antibodies at low titer. Some of the youngest relatives exhibited immunological abnormalities and they may be high-risk subjects with regard to Waldenstrom's macroglobulinemia.

Aged↗