Sclerosing angiomatoid nodular transformation of the spleen associated with thrombocytopenia.

Sclerosing angiomatoid nodular transformation of the spleen, a recently described lesion of unknown pathogenesis, with a benign clinical course that is very often asymptomatic. Sclerosing angiomatoid nodular transformation may be a novo lesion or the final common pathway of various benign splenic conditions such as hamartoma, inflammatory pseudotumor and hemangioma. We report the case of a 68 year-old woman with thrombocytopenia and a splenic mass, diagnosed as sclerosing angiomatoid nodular transformation.

Vinorelbine and prednisone in frail elderly patients with intermediate-high grade non-Hodgkin's lymphomas.

BACKGROUND: Frail patients with non-Hodgkin's lymphoma (NHL) are generally excluded from clinical trials and not even treated. The aim of this study was to evaluate the efficacy and tolerability of vinorelbine and prednisone in frail elderly patients with NHL. PATIENTS AND METHODS: Thirty consecutive frail elderly patients were entered in a phase II study with vinorelbine 25 mg/m2 i.v. on days 1 and 8 and oral prednisone 30 mg total dose on days 1-8 for six cycles. Criteria of frailty were age > or =80 years, or age > or =70 years and three or more comorbidities of grade 3 or at least one comorbidity of grade 4 according to the Cumulative Illness Rating Scale (CIRS), or not self-sufficient or the presence of one or more geriatric syndromes. RESULTS: Of 30 evaluable patients, three (10.0%) achieved a complete response (CR), nine (30.0%) showed a partial response (PR), while 10 presented with stable disease and eight with progressive disease. The median duration of CR was 29 months (range 5-36 months), and the median duration of PR was 1 month (range 1-22 months). Three patients had grade 3 neutropenia and one had grade 4. One grade 4 neurotoxicity was observed. Three patients died because of heart failure within 28 days of therapy, and one patient died after 4 days because of rapid progression. The median overall survival was only 10 months. CONCLUSION: Vinorelbine and prednisone is a relatively non-toxic combination with modest activity in frail patients with NHL. If initial aggressive chemotherapy has been excluded, this combination could be tried to obtain a temporary palliation.

Severe bleeding due to acquired hypoprothrombinemia-lupus anticoagulant syndrome. Case report and review of literature.

A 17-year-old girl was admitted to our department with a hemorrhagic syndrome due to a serious coagulopathy; prothrombin time (PT) INR was 2.46 and the activated partial thromboplastin time (aPTT) ratio 3.46. Coagulation tests with pooled normal fresh plasma did not correct aPTT because of a coagulation inhibitor, and only partially corrected PT. Factor II activity reached only 5%. Diluted Russell viper venom tests (dRVVT) and kaolin clotting time (KCT) of patient plasma (PP) and of a mixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). The level of factor II antigen was 10%. We diagnosed systemic lupus erythematosus (SLE) with a rare acquired hypoprothrombinemia-LA syndrome (HLAS). The patient was treated with corticosteroids and high-dose Ig and a normal PT value was re-established.

[Is early diagnosis of amyloidosis AL possible? Consideration on a representative case]. / Esiste la diagnosi precoce della amiloidosi AL? Considerazioni su un caso emblematico.

A male patient, 53 year-old, affected by with amyloidosis AL (AAL) with MGUS IgG lambda shows the renal function and the spiro-index yet normal. There is a cardiomyopathy by amyloidosis, but the kinaemia is yet normal. We think to treat the patient with high dose therapy and PBSCs. The appearance of a heart failure and an asystole after the anaesthesia of marrow-harvest precludes this treatment: once more the diagnosis of amyloidosis AL was late. Is the early diagnosis of AAL possible?

[Di George's syndrome with neutropenia in an adult patient]. / Sindrome di Di George con neutropenia in un paziente adulto.

The clinical data of a thirty-nine old inpatient woman are reported, whose main complaints were non-operable vulvo-vaginal condylomata, recurrent bacterial infections, complicated chickenpox and prominent lymphopenia. The peculiar facies get us to suggest the diagnosis of a case of the Di George syndrome in an adult patient. Was probably the associated neutropenia congenital and combined with immunodeficiency syndrome?

[A case of resistant sideropenic anemia. Adult celiac disease]. / Un caso di anemia siderocarenziale resistente. Malattia celiaca dell'adulto.

We report a case of a woman affected by sideropenic anaemia (SA) for about thirty years and never restored with a therapy with iron per os. At the age of 60 years a malabsorption syndrome appears and an adult celiac disease was diagnosed (ACD). The SA was therefore and since the beginning the only symptom of the malabsorption. When SA is resistant to the iron per os therapy, we always should suspect a malabsorption due to CD often olygosymptomatic in the adult patient. We would now recommend, in presence of resistant SA, the weight of jejunum biopsy, as well as his excessive importance, when it shows an apparently not specific finding of mucosa atrophia with chronic phlogosis.

[Acute kidney failure in the patient with multiple myeloma. An analysis of the authors' personal cases]. / L'insufficienza renale acuta del paziente con mieloma multiplo. Analisi di una casistica personale.

The renal involvement in a multiple myeloma case (MM) has a frequency of 50% and causes a worsening of the disease with a survival average of about 12 months. Myeloma cast nephropathy (MCN) represents the more frequent clinic, histological form of nephropathy in course of MM and it evolves when monoclonal light free chain deposit in the renal tubules together with some other worse cases like dehydration and/or hypercalcaemia. We analyze here the clinical and renal histological features of eight patients treated for acute renal failure found in MCN in course of MM grade B. This was discovered through renal bioptic check-up. We have evaluated the Bence-Jones proteinuria, the recurrence of the condition of risk and the course of the renal failure of these patients also in order to treat the hematological illness.

A "primitive" catastrophic antiphospholipid syndrome.

A female patient affected by a thrombotic syndrome due to non SLE-related antiphospholipid antibodies (APA), developed a serious, non inflammatory, thrombotic macroangiopathy and uremic-hemolytic syndrome during oral anticoagulant therapy. She was treated with aspirin, dipyridamole i.v. and with a total of 14 fresh frozen plasma exchange (PEX), but the thrombotic syndrome only showed a partial response with the APA titre dropping considerably. Renal failure did not improve because of acute cortical necrosis. The patient died from infective complications.

[The kidney in multiple myeloma. The physiopathological and clinical aspects]. / Il rene nel mieloma multiplo. Aspetti fisio-patologici e clinici.

The renal concern in a multiple myeloma (MM) case has a frequency of 50% and causes a worsening of the disease with a survival average of about 12 months. The monoclonal light free chains (CLL) produced in excess by the plasmacytes are present in the urine as proteinuria of Bence Jones (PBJ) in 60-70% of patients affected by MM. They represent the major pathogenetic factor of the nephropathy in course of MM as they can deposit in shape of intratubular "casts" in the myeloma casts nephropathy (MCN). In some worse cases, dehydration or hypercalcaemia can cause an irreversible acute renal insufficiency (RI). It is therefore important in a patient affected with MM with PBJ to prevent, locate and opportunely treat these situations which worsen the nephropathy. Beside the tubular cast nephropathy, the CLL "accumulate" in the kidney even though with a lower frequency compared to MCN, in the light chains deposition disease (LCDD) and in the amyloidosis AL (AL). LCDD is characterized by a deposit of nodular amorphous materials PAS positive in the glomerulus and sometimes even in the tubulus. It usually presents itself as a chronic RI and a proteinuria causing nephrotic syndrome (NS). This quickly evolves into uraemia and its evolution can be lessened by the MM treatment. AL in course of MM also reveals with a chronic RI and NS. CLLs deposit in the typical fibrillar structure, on the vessel walls, in the glomerulus, in the mesangium and can be marked out with the Congo red colouring and the subsequent green birefringence through microscope with polarized light. Prognosis of AL is extremely severe and no benefit is given by the treatment of the hematological illness. It is therefore absolutely necessary to study the renal histology through biopsy when MM is grade B, that is, with serumal creatinine above 2 mg/dl as: MCN imposes the MM treatment programme in order to reduce the tubular excess of PBJ and to attempt to make RI reversible; MCN with tubular atrophy and interstitial fibrosis results in an unfavourable prognosis as it expresses a nephropathic irreversibility due to the loss nephrons. It will therefore necessary to start on a renal substitutional treatment programme. Renal damage in course of MM is not always tubular, rather an unexpected glomerular damage of LCDD or amyloidosis AL type can be found.(ABSTRACT TRUNCATED AT 400 WORDS)

A hemorrhagic syndrome of the elderly patient caused by anti-factor VIII antibodies.

We report here 2 cases of elderly patients who were admitted to our Department of Geriatrics for a hemorrhagic syndrome. The patients were found to be affected by a coagulopathy with prolonged activated partial thromboplastin time (aPTT), caused by the presence of an acquired inhibitor of Factor VIII. This syndrome, due to anti-Factor VIII antibodies, is surely an important clinical entity that is not easy to diagnosed, but in concomitance with a hemorrhagic syndrome and prolonged aPPT it should always be suspected. In fact, a quick diagnosis is very important in order to apply specific treatment as soon as possible.

Prolymphocytic leukemia: a very satisfactory response to treatment with recombinant interferon alpha.

We report the case of a patient with prolymphocytic leukemia in whom a lengthy survival of 5 years was observed after treatment with splenic irradiation and chemotherapy. The patient obtained a surprising improvement with a significant reduction in the absolute count of the prolymphocytes and considerable reduction in splenomegaly after 3 months' therapy with interferon alpha.