Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis

Persistent polyclonal B cell lymphocytosis (PPBL) is a rare lymphoproliferative disorder of unclear natural history and its potential for B cell malignancy remains unknown. We describe the case of a 39-year-old female who presented with stage IV-B large cell lymphoma 19 years after an initial diagnosis of PPBL; her disease was rapidly fatal despite intensive chemotherapy and blood stem cell transplantation. Because we had recently identified multiple bcl-2/Ig gene rearrangements in blood mononuclear cells of patients with PPBL, we sought evidence of this oncogene in this particular patient: bcl-2/Ig gene rearrangements were found in blood mononuclear cells but not in lymphoma cells. Owing to the possible role of Epstein-Barr virus (EBV) in the pathogenesis of PPBL, we also hypothesized our patient might have an EBV-related lymphoproliferative disorder. Despite serologies consistent with past exposure to this virus, it was not found in lymphoma cells using a sensitive polymerase chain reaction technique. We conclude that non-Hodgkin's lymphoma may occur during the course of PPBL. However, longer follow-up in more patients will be needed in order to better clarity the risk of hematologic malignancy in patients with PPBL.     

Severe encephalopathy in a child: an uncommon cause

We report the unusual fixed cutaneous variety of sporotrichosis in a patient hailing from Uttarkashi (Uttar Pradesh) in the north-western part of India.     

Car surfing: an uncommon cause of traumatic injury

Car surfing is an infrequent cause of traumatic injuries treated by emergency physicians. This very dangerous activity can result in serious injury or death. We report 5 cases of injuries caused by car surfing seen at our hospital during 1996 and 1997. All involved head injuries after a fall from a moving motor vehicle. There were 3 male and 2 female patients, and 3 cases were fatal. Health care providers should be aware of this type of injury and support efforts to prevent it.     

Lambl's excrescence: an uncommon cause of cerebral embolism

A 31 year old right handed woman presented with acute onset of aphasia which cleared over two days. CT-scan showed a left middle cerebral artery infarct within Wernicke area. Initial transesophageal two-dimensional echocardiography disclosed a mitral valve lesion suggesting a thrombus. She was discharged on oral anticoagulant treatment. A second stroke occurred ten months later involving left lenticulo-striate arteries area. Echocardiography remain unchanged. Subsequently, giant Lambl's excrescences of mitral valve was confirmed by operation and pathologic examination. The majority of patients with Lambl's excrescences are asymptomatic. However surface thrombus is common with this tumors which reposant a potential us for cerebral embolization. These tumors should be operated since complete excision is the only definitive means of eliminating the source of recurrent embolization.     

Neoplastic pulmonary embolism. An uncommon cause of acute respiratory distress with normal pulmonary radiography

BACKGROUND: Ocular pulse amplitude (OPA) is reduced in normal tension glaucoma (NTG) patients when compared to non-glaucomatous, healthy control subjects. This might be related to a vasospastic reaction. The objective of this study was to determine if low OPA in NTG is associated with a vasospastic reaction and its response to vasodilation. METHODS: Nifedipine, a calcium channel blocker, vasodilator and systemic antihypertensive agent improves visual fields in NTG patients following acute and chronic dosing. The effect of 60 mg of daily orally administered nifedipine on OPA, intraocular pressure (IOP, German abbreviation: IOD), blood pressure (BP, German abbreviation: RR) and pulse rate (PR, German abbreviation: HF) were measured prior to and for 3 months after initiating nifedipine therapy in 32 NTG patients with and without a vasospastic reaction as manifested by a local cold exposure test. Before treatment, all patients had reduced OPA evaluated with the Langham Ocular Blood Flow System. RESULTS: During nifedipine treatment NTG patients with a vasospastic reaction showed a significant (p < 0.001) increase in OPA, whereas NTG patients without a vasospastic reaction showed no sig. (p > 0.05) change in OPA. CONCLUSION: There may be two different subgroups of NTG patients, those who have a vasospastic reaction and react to nifedipine, while others lack the ability to react to nifedipine or might have a different, non-vasospastic pathology. Calcium channel blockers and other vasodilators may be useful in the treatment of vasospastic NTG patients.     

Aggressive NK cell lymphoma preceded by a ten year history of neutropenia associated with large granular lymphocyte lymphocytosis

We report a case of aggressive natural killer (NK) cell lymphoma in an 82 year old man who first presented 10 years earlier with neutropenia in association with a large granular lymphocyte (LGL) lymphocytosis. The diagnosis of NK cell lymphoma was made on the basis of morphological and immunological characteristics (CD3-CD56+) found on skin biopsy of one of multiple skin nodules which subsequently developed in association with splenomegaly, thrombocytopenia and continuing neutropenia. In addition there was BM infiltration and a cytogenetic abnormality [add(6)(p25)] was detected. Combination chemotherapy led to an initial clinical response but a relapse occurred shortly afterwards and the patient died 8 months later from infection whilst neutropenic following re-introduction of chemotherapy. Previously reported cases of aggressive NK cell lymphoma have shown a young male predominance with a rapidly progressive clinical course and without evidence of a preceding chronic phase of LGL lymphocytosis and neutropenia.     

Arteriovenous malformations of the uterus: an uncommon cause of vaginal bleeding

Arteriovenous malformations (AVM) are rare entities in gynecology, with only 73 cases reported in the literature. Most commonly they present with vaginal hemorrhage, but other presentations such as congestive heart failure, postmenopausal bleeding, and an asymptomatic mass have been described. These lesions may be congenital or acquired. Acquired lesions are believed to follow trauma or may arise after choriocarcinoma or other gynecologic malignancies. Diagnosis can rapidly be made with color flow Doppler ultrasound or angiography. Additionally, they have been detected using hysteroscopy, hysterosalpingogram, and computerized tomography. Acute management consists of hemodynamic stabilization and possibly placement of a Foley bulb in the uterus or methylergonovine injection. Ultimate treatment depends on the patients desire for fertility. Embolization therapy is variably successful and may allow the preservation of reproductive capacity. To date, five pregnancies after embolization have been reported with varying outcomes. If pregnancy is not desired or embolization fails, hysterectomy remains the treatment of choice.     

Splenic marginal zone cell lymphoma associated with clonal B-cell populations showing different immunoglobulin heavy chain sequences

Splenic marginal zone cell lymphomas (SMZCLs) are low-grade B-cell lymphomas that usually present with massive splenomegaly and subtle (subleukemic) peripheral blood involvement. Polymerase chain reaction (PCR) analysis of peripheral blood from a patient with subleukemic SMZCL showed evidence of two clonal immunoglobulin heavy chain (IgH) gene rearrangements. IgH PCR analysis of DNA derived from the patient's splenic neoplasm demonstrated a single clonal IgH rearrangement, which had a different electrophoretic mobility from either of the two PCR products detected in the patient's peripheral blood. Additional characterization of these PCR products by DNA sequencing demonstrated two independent IgH rearrangements in the peripheral blood, one of which used IgH joining region 6c (JH6C) and the other JH4. A different IgH rearrangement was present in the splenic tumor, which used JH4a. No sequences from the splenic neoplasm were detected in the peripheral blood and vice versa. This case illustrates that PCR might reveal monoclonal populations in peripheral blood unrelated to the presence of lymphoma in other anatomic compartments.     

Splenic angioma with thrombocytopenia

A case of splenectomy with thrombocytopenia but without hypersplenism is described. The etiology, proven by splenectomy, was a splenic angioma. The isotopic examinations necessary to localize the site of a vascular tumor and the relations between thrombocytopenia and hemangioma are discussed.     

Splenic lymphoma with villous lymphocytes and Waldenstrom disease: diagnostic difficulties by way of a case report

A 67-year-old man visited our hospital with complaints of scrotal swelling associated with occasional febrile episodes. Physical examination disclosed a huge scrotal mass, approximately the size of a child's head, with numerous papillomatous lesions on its surface. His past medical history was significant in that he was diagnosed with penile carcinoma at the age of 35 years old and was treated with partial penectomy followed by radiation and chemotherapy at other hospital. During this admission tumor marker squamous cell carcinoma (SCC) and microbiological tests for mcroflariae were both negative. Ultrasound (US), computed tomographic (CT) scan and magnetic resonance imaging (MRI) revealed markedly thickened scrotal skin and small hydrocele with no evidence of local recurrence of the previous penile carcinoma. A percutaneous cystostomy was created because of chronic urinary retention and possible urine extravasation into the scrotum. Histopathological examination of the biopsy specimen from the scrotal mass demonstrated lymphangiectasia consistent with elephantiasis of the scrotum. Surgical excision of this huge scrotal mass was performed in August 1997. The resected tissue weighed 1,400 g. Convalescene was uneventful. He subsequently underwent perineal urethrostomy in place of the suprapubic cystostomy.     

Primary aldosteronism with uncommon complications

In a patient who had primary aldosteronism and severe total-body potassium depletion muscular tonic contractures developed during induction of anesthesia. After correction of the potassium deficit, the patient underwent uneventful anesthesia and transabdominal right adrenalectomy. Neither serum potassium level nor EKG seems to provide a reliable index of correction of potassium deficit. Measurement of potassium balance provided a method of quantitating the potassium depletion and of determining when the potassium deficit had been corrected. Balance studies should be utilized preoperatively when long-term potassium loss is suspected to reduced complication secondary to hypokalemia.     

Aortopulmonary fistula in an infected Dacron graft for coarctation of the aorta: an uncommon cause of hemoptysis

Hemoptysis secondary to an aortobronchial fistula is rare and uniformly fatal when left untreated. The authors describe a case of massive hemoptysis caused by an aortopulmonary fistula in an infected Dacron graft used successfully to repair a coarctation of the aorta.     

Splenic hemangiomatosis with osseous involvement

A rare case of splenic hemangiomatosis with bone involvement in the upper extremity is presented. The findings on ultrasonography and CT scan abdomen were suggestive of splenic hemangiomatosis. X-ray of left forearm showed findings characteristic of osseous hemangiomatosis, which was subsequently confirmed on histology.     

Incidence of bacteremia associated with chorionic villus sampling

OBJECTIVE: To assess the frequency of transient bacteremia among women undergoing transabdominal and transcervical chorionic villus sampling (CVS). METHODS: One hundred fourteen women undergoing CVS consented to participate in a university review board-approved study protocol. Exclusion criteria included known cardiac valve anomaly or replacement (or other prosthetic) and antibiotic use within the preceding 21 days. Blood cultures (aerobic and anaerobic) were drawn by a single operator on all patients, before CVS and within 15 minutes after completing CVS. Either the catheter tip or needle tip aspirate from each procedure was also sent for culture. RESULTS: Post-procedure bacteremia was detected in two (1.8%) of the patients undergoing CVS. These two patients both had their procedures performed transcervically, resulting in a 4.1% (two of 49) bacteremia rate after transcervical CVS, compared to none (zero of 65) in the transabdominal group (P = .36). The incidence of positive cultures from sampling instruments was also higher in the transcervical group (16.3 versus 0%; P = .003), but did not result in comparable rates of bacteremia among patients with positive instrument cultures. CONCLUSIONS: In this study, CVS was associated with a low rate of bacteremia, regardless of the procedure route. Recommendations for antibiotic prophylaxis in women with abnormal cardiac valves should parallel those for spontaneous vaginal delivery and other comparable genitourinary procedures.     

Primary pancreatic lymphoma. A rare cause of pain-free icterus

Toxocariosis is a zoonosis which has been widely studied in dogs. However, not much is known about this parasitosis in cats. The aim of the present work was to determine the frequency of Toxocara cati in domestic cats in Mexico City and the State of Mexico. Feces of 660 domestic cats were studied by the Faust concentration-floating test; 401 samples were from cats living in Mexico City of which 308 lived in houses and 93 in apartments; 231 were from urban areas of the State of Mexico and 28 from a rural area of the same state. The total frequency of T. cati eggs obtained from domestic cats in Mexico City was 42.9%; in cats living in apartments it was 18.3% and in cats living in houses it was 50.3%. In domestic cats from the State of Mexico, T. cati frequency was 36.4% in the urban and 21.4% in the rural areas. We consider that toxocariosis frequency observed at the two studied sites is high and that the need to prevent dissemination of the infectious forms of T. cati is urgent, as is the necessity of informing the population of the risk of living with T. cati parasited animals and of the anatomopathological alterations caused by T. cati in man.     

Splenic irradiation for hairy cell leukaemia

In vitro studies show that the adhesive amalgam technique is superior to the nonadhesive technique. Also, early clinical results indicate that the adhesive technique can eliminate the microspace between amalgam and tooth. And, it can retain amalgam on unprepared occlusal surfaces of molars and premolars, sealing the fissures. Moreover, amalgam can be retained in preparations without undercuts. Early results indicate in traditional preparations, the adhesive technique appears to be at least equivalent to nonadhesive technique.