Bisalbuminemia en un paciente con un cáncer de pulmón diseminado / Bisalbuminemia in a patient with metastatic lung cancer

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Fever of unknown origin (FUO) in an immunocompetent adult due to cytomegalovirus (CMV) with polyclonal gammopathy.

Fever of unknown origin (FUO) may be due to infection, malignancy, collagen vascular/inflammatory disorders, or other causes. Cytomegalovirus (CMV) infection is a rare cause of FUO in immunocompetent adults. We present a case of FUO due to CMV in an immunocompetent adult with polyclonal gammopathy on serum protein electrophoresis (SPEP).

Coagulopathies and arterial stroke.

Although hypercoagulable states are most often associated with venous thrombosis, arterial thromboses are reported in protein S, protein C, and antithrombin III deficiencies, factor V Leiden and prothrombin gene mutations, hyperhomocysteinemia, dysfibrinogenemia, plasminogen deficiency, sickle cell disease, and antiphospholipid antibody syndrome.

Recurrent retinal vein occlusion in a patient with increased plasma levels of histidine-rich glycoprotein.

PURPOSE: To report an association between retinal vein occlusion and increased plasma levels of histidine-rich glycoprotein. DESIGN: Observational case report. METHODS: A 56-year-old woman presented with sudden and painless decrease of visual acuity of her right eye. Indirect ophthalmoscopy revealed retinal vein occlusion. She had experienced central retinal vein occlusion in this eye 6 years earlier. RESULTS: The patient's medical history was negative for cardiovascular risk factors. Further investigation into possible causes revealed increased values for histidine-rich glycoprotein. CONCLUSIONS: When ordering laboratory tests in patients with retinal vein occlusion to rule out coagulation disorders, increased plasma levels of histidine-rich glycoprotein should be considered in the differential diagnosis. Selective screening may be helpful in identifying retinal vein occlusion patients with thrombophilic defects, especially in young individuals with recurrent retinal vein occlusion in the absence of recognized cardiovascular risk factors.

Oral lesions indicative of plasminogen deficiency (hypoplasminogenemia).

BACKGROUND: Gingival overgrowth with ulceration has recently been recorded in 4 reports: (1) our report of a British patient with ligneous conjunctivitis in whom the gingival lesions appeared to be related to tranexamic acid-an antifibrinolytic agent; (2) a report of 2 Turkish patients and an Italian patient with mainly gingival lesions; (3) our report of 5 Turkish patients with mainly gingival lesions; and (4) a report of 3 new Turkish cases, which also were associated with gingival lesions and alveolar bone loss. These patients all had gingival swellings, and a minority had conjunctival involvement similar to ligneous conjunctivitis, although the etiology was unclear in all. Nevertheless, fibrin exudation was fundamental because the hyaline or amyloidaceous material seen on the gingival biopsy stained for fibrin but failed to stain for amyloid. METHODS: We have examined 6 more patients who exhibited gingival swelling caused by amyloidaceous deposits that stained only for fibrin, and we assayed their plasminogen levels. RESULTS: The plasminogen functional activity assayed in these 6 additional patients, and in 2 of the 5 patients previously reported by us, was significantly reduced. CONCLUSIONS: Gingival overgrowth with ulceration appears to be a new complication caused by plasminogen deficiency; it also appears to be related to ligneous conjunctivitis in some cases.

Cytoplasmic cytokines in lymphoproliferative disorders: multiple cytokine production in angioimmunoblastic lymphadenopathy with dysproteinemia.

Cytokines play an important role in the pathogenesis of lymphomas via autocrine or paracrine mechanisms, or both. Here we determined the proportion of CD3-positive T lymphocytes containing various types of cytokines in enlarged lymph nodes. Lymph nodes were obtained from 16 patients with various lymphoproliferative disorders, including 3 cases with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD), 3 cases with adult T cell leukemia/lymphoma (ATLL), 2 cases with T-cell nonspecific malignant lymphoma (T-ML), 3 cases with B-cell diffuse large malignant lymphoma (BDL), 3 cases with histiocytic necrotizing lymphadenitis (HNL), and 2 cases with non-specific lymphadenitis (NSL). The percentages of T lymphocytes positive for cytoplasmic cytokines IL-2, IL-4, IL-5, IL-6, IL-13, TNF-alpha, and INF-gamma were determined. The percentage of INF-gamma positive T lymphocytes was high in reactive lymphadenopathy of NSL and HNL. AILD showed a high proportion of TNF-alpha positive T-lymphocytes, and in addition, the percentages of IL-2, IL-4, IL-5, IL-6, IL-13 and INF-gamma positive T-lymphocytes were relatively higher than in other diseases. Our results supported the state of multiple hypercytokinemia typically seen in AILD and suggested that the source of the cytokines is the lymph nodes. Our results also suggested that multiple cytokine networks play an important role in the clinical and histopathological features of AILD. Modulation of the cytokine network may be the logical objective in future therapeutic strategies designed for AILD.

Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia: a clinical, immunohistological, and molecular analysis.

OBJECTIVE: To determine whether cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is related to a clonal T-cell proliferation. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Ten patients with AILD and cutaneous involvement. MAIN OUTCOME MEASURE: The T-cell receptor-gamma (TCRG)gene rearrangement was studied with the use of polymerase chain reaction and denaturing gradient gel electrophoresis in blood, nodal, and skin samples. Skin and nodal samples were investigated also for the presence of Epstein-Barr virus (EBV) RNA by in situ hybridization. RESULTS: A transient morbilliform eruption of the trunk was seen most often. Other cutaneous features were infiltrated plaques and purpuric or urticarial lesions. A clonal TCRG gene rearrangement was detected in 7 skin samples, corresponding to a maculopapular eruption with a histological pattern of nonspecific mild lymphoid dermal infiltrate in 6 patients, and to erythematous plaques with histological findings of typical cutaneous lymphoma in 1 patient. In the 5 patients in whom a TCRG gene rearrangement was evidenced in skin and lymph node samples, identical clones were detected in both. Five patients died by the end of the study, with a mean survival of 33.2 months. Four of these 5 patients had a clonal infiltrate in skin and lymph nodes. The EBV RNA was detected in only 1 of 10 skin biopsy specimens and in 5 of 8 lymph nodes tested. CONCLUSIONS: Cutaneous involvement is often related to a clonal T-cell proliferation in AILD, even when clinical and histological features are nonspecific. Cutaneous infiltrate seems to be clonally related to the nodal T-cell proliferation. The role of EBV infection in skin lesions was not evidenced.

Seropositive rheumatoid arthritis with dermatomyositis sine myositis, angioimmunoblastic lymphadenopathy with dysproteinemia-type T cell lymphoma, and B cell lymphoma of the oropharynx.

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder that often progresses to high grade T cell lymphoma. We describe a 63-year-old woman with longstanding seropositive rheumatoid arthritis who developed fever, cutaneous findings of dermatomyositis, a diffuse pruritic maculopapular rash, enlarged lymph nodes, polyclonal elevated serum gammaglobulins, and an IgG lambda paraprotein. Lymph node biopsies yielded tissue with characteristic changes of AILD and T cell lymphoma. Interleukin 6 (IL-6) was present during the early, active phase of disease, and circulating IL-6 and IL-2 were detected one month before tumor recurrence. Two years after AILD and T cell lymphoma were diagnosed, she developed a B cell lymphoma that involved the oropharynx.

A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) together with total atelectasis of a lung caused by lymphadenopathy and pleural effusion.

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a clinicopathologic entity established by Frizzera in 1974. Reported cases of AILD with pleuropulmonary involvement have been increasing recently. At Nogata Central Hospital, an 82-year-old male inpatient with brain infarctions and chronic bronchitis showed a rapid growth of systemic lymphadenopathy and various other symptoms. We diagnosed the case as AILD by histopathologic examination of a lymph node. Chest radiography and computed tomography demonstrated a loss of volume of the right lung caused by intrathoracic lymphadenopathy and a pleural effusion. Although cases of AILD with pleuropulmonary involvement have been increasing, no cases with almost total atelectasis of a lung have been reported as yet. AILD should be taken into account as a disease which may cause atelectasis of a lung.

Successful treatment of angioimmunoblastic lymphadenopathy with dysproteinemia by local irradiation.

We report an 85-year-old man presenting with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) of the palatine tonsil with cervical and axillary lymph node enlargement, pleural effusion and hypergammaglobulinemia. The patient was treated in Internal Medicine with combined chemotherapy with steroids which resulted in a complete remission. However, he returned almost 1 year later with an ulcerative lesion in the left palatine tonsil, with no lymph node involvement. Considering that the patient was an elderly person, chemotherapy had been administered during the initial admission, tumor cells appeared to originate from T cells and relapse occurred only in the local lesion, the lesion was treated by local irradiation using 60Co at a dose of 40 Gy, which produced a complete resolution. Radiotherapy appeared to be effective for the treatment of localized lesions of AILD.

A straightforward venous ulcer. Or is it?

Venous ulcers are common in clinical practice. They are due to end stage skin and subcutaneous damage from sustained venous hypertension. The common cause may be post thrombotic syndrome or primary superficial venous insufficiency. This case illustrates the need to think of inherited thrombophilia as a primary cause.

Morphometric evaluation of paraneoplastic neuropathies associated with carcinomas, lymphomas, and dysproteinemias.

Paraneoplastic peripheral neuropathies are caused by indirect effects of carcinomas, mainly small cell bronchogenic carcinomas, lymphoproliferative disorders (lymphomas, myelomas, polycythemia vera), and dysproteinemias (benign monoclonal paraproteinemia, Waldenström's macroglobulinemia) including cryoglobulinemias. Those associated with carcinomas are usually considered as severe, those associated with benign gammopathies (monoclonal gammopathies of unknown significance, MGUS) as mild, and those with cryoglobulinemias as of variable severity. In a larger series of 104 autopsy and biopsy cases, we noted a wide range of severity concerning various morphometric parameters of peripheral nerve fibers by evaluating sural nerves. There were no apparent morphometric differences between the groups of disorders. The most valuable parameter of optic-electronic evaluation and comparison turned out to be the myelin area expressed as a percentage of the endoneurial area because this measure comprises the relative number, size, and myelin thickness of the myelinated nerve fibers. In the 104 cases of the three disease groups, most of the cases (38 cases; 36.5%) showed a moderate reduction of the myelin sheath area per endoneurial area of sural nerves. This was followed by 34 cases (32.7%) with severe and very severe reduction. Twenty-nine cases (27.9%) presented with mild reduction. It is concluded that the severity of the neuropathy depends largely on the stage of the disease and the time of progression rather than on the type of the underlying disorder.

Angioimmunoblastic lymphadenopathy with dysproteinemia--lack of a prognostic value of clear cell morphology.

It has been suggested that angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is closely related to peripheral T cell lymphoma (PTCL). However, the clinical course of AILD-like PTCL is notoriously unpredictable. A minor portion of patients enjoyed prolonged remission with steroid-only treatments (indolent AILD) while most others died rapidly despite the use of intensive chemotherapy (aggressive AILD). Recently, it has been suggested that histological features such as the presence or absence of clear cells and convoluted cells are of high prognostic value. The validity of this observation was addressed in this study. Eighteen patients who presented between 1977 and 1994 at the National Taiwan University Hospital were retrospectively studied. There were 11 men and 7 women, with a median age of 47 years. Twelve patients had received various regimens of systemic chemotherapy, and the other 3 patients had been treated with steroids alone. Eight patients had indolent AILD and 6 aggressive AILD. The follow-up period in 4 patients was too short to be analyzed. The histopathology of these cases was divided, according to the criteria of Aozasa et al., into group I (neither cells), 4 patients; group II (only convoluted cells), 1 patient, and group III (clear cells with or without convoluted cells), 13 patients. Contrary to others, our data revealed that group III patients were doing better than group I patients. Univariate analysis of other pertinent clinical features, including sex, age, lymphadenopathy, B symptoms, hepatosplenomegaly, hypergammaglobulinemia, elevated serum lactate dehydrogenase, and treatment regimens, revealed none of them to be prognostically relevant. However, patients who had achieved complete remission by steroids or other systemic chemotherapy had a significantly better prognosis than those who had not. Together, these preliminary data suggested that (1) the presence or absence of clear cells and convoluted cells failed to predict the clinical behavior, and (2) induction of complete remission by steroids or other chemotherapeutic agents is an important prognostic index.

Activated protein C resistance in homozygous sickle cell disease.

The activated protein C resistance (APC-R) ratios in 50 patients with steady state homozygous sickle cell (SS) disease and 59 healthy AA controls was measured. There was a significant reduction in median APC-R ratio in sickle cell disease compared to controls. This reduction in APC-R ratio was not explained by (1) the presence of the factor V Leiden, found in only one of 165 patients with SS disease including those tested for APC-R, or (2) the presence of lupus anticoagulants. However, the raised levels of factor VIIIC in SS patients in this study may be contributing to increased resistance to APC, which in turn may contribute to the vaso-occlusive complications of SS disease.