Plasma Cell Leukaemia Masquerading as Hairy Cell Leukaemia: A Case Report

Plasma cell leukaemia is a rare and aggressive neoplasm with survival of less than one year with conventional treatment. It can rarely present with morphology mimicking hairy cell leukaemia. We present a case of plasma cell leukaemia with hairy cell morphology for its rarity, diagnostic difficulty and aggressive course.     

Hairy Cell Leukemia: Clinicopathological and Immunophenotypic Study

Hairy cell leukemia (HCL) is a rare neoplasm of mature small B lymphoid cells with characteristic circumferential ‘hairy projections’ involving the peripheral blood, bone marrow and splenic red pulp. With the advent of immunophenotyping and newer treatment modalities, prolonged remission can be achieved after a definitive diagnosis. Due to the rarity of this condition and presence of only a few case series from India, this work was undertaken. The aim was to study the clinico-pathologic and immunophenotypic features of all cases diagnosed as hairy cell leukemia. The cases were retrieved from Hematopathology records, between 1991 and 2012. The complete clinical details, investigations, treatment and follow-up were obtained from Medical Oncology records. The peripheral blood picture, bone marrow cytology and trephine sections along with special stains were reviewed. There were 12 cases of HCL during the study period with a M:F ratio of 11:1. Of these, ten were diagnosed as classical HCL and two as variant HCL. The most common clinical manifestations were fever, easy fatigability and weakness. Splenomegaly was present in 81.8 % cases. Though all the patients showed some form of cytopenia, there were three (25 %) patients with leucocytosis. The smears from all patients showed atypical lymphoid cells with circumferential hairy projections. TRAP was positive in 9 patients (81.8 %). Immunophenotyping was done in six cases, four were confirmed as HCL and two were diagnosed as HCL-v. The patients treated with Cladribine generally had a good response. The characteristic morphology of the hairy cells; along with correlation with the clinical features, TRAP positivity and immunophenotyping by flow cytometry is essential for diagnosis. Treatment response with Cladribine is good and has prolonged remission rates.     

Atypical Mycobacterial Infection in Hairy Cell Leukemia Treated with Cladribine

We report a case of hairy cell leukemia who improved after cladribine but succumbed to disseminated atypical mycobacterial infection 2 months after completing cladribine.     

Sex Differences in Infective Endocarditis

BackgroundThe impact of sex on the presentation, etiology, and outcomes of infective endocarditis (IE) has not been adequately studied. The aim of the present research was to analyze the impact of sex on the presentation, etiology, and outcomes of IE.MethodsWe performed a retrospective study of 214 adult patients (131 male and 83 female) with IE. All cases of IE were reviewed by two investigators- both senior physicians in internal medicine. Two groups of patients were compared: male and female patients with IE. The primary outcome was in-hospital mortality.ResultsWe found significant differences in etiologic factors of IE in male and female patients. Microbiologic etiology differences between male and female groups of patients were in coagulase negative staphylococcus (15.0% in male vs 3.8% in female groups, P = 0.011), and culture negative endocarditis (8.7% in male vs 23.8% in female groups, P = 0.004). We did not find a difference in the primary outcome between the two groups; however, all-cause mortality was significantly higher in the female group as compared to the male group (26 [31.3] vs 22 [16.8], P = 0.018).ConclusionsWe found that sex may have important role in both the microbial profile and the patient's outcome with IE.     

感染性心内膜炎的外科治疗

目的：探讨感染性心内膜炎（ＩＥ）的诊断及外科治疗。方法：我院从１９８６年１１月至１９９６年５月，外科治疗感染心内膜炎患者１６例，其中男性１２例，女性４例。手术方法：全麻低温体外循环急诊换瓣手术７例（主动脉瓣替换５例，主动脉瓣＋二尖瓣替换１例，主动脉瓣替换＋膜部心室间隔缺损涤沦补片修补１例）；择期换瓣手术９例（主动脉瓣替换７例，二尖瓣替换２例）。切除瓣周感染组织，对散在于心室间隔和腱索上难以切除的微小赘生物电灼，术毕抗生素溶液冲洗心腔。结果：全组１６例。急诊手术７例，其中术后死亡１例（死亡率１４．３％），死亡原因为多器官衰竭；择期手术９例，无手术死亡。结论：反复多次血培养结合超声心动图检查，可使ＩＥ诊断阳性率大大提高。尽早手术是对部分ＩＥ患者治疗的基本原则，无法控制的感染和心力衰竭是尽早手术治疗的最佳适应证。     

Outpatient Management of Infective Endocarditis

Because of unique host defense characteristics of the endocardium, successful therapy for infective endocarditis (IE) has necessitated bactericidal antimicrobial agents, generally administered in high doses for prolonged periods of time. This has required therapy in the hospital setting. However, in recent years, it has become apparent that outpatient therapy is feasible if appropriate agents can be administered at home, for example, using either home parenteral therapy or oral preparations. Over the past few decades, there has been a trend toward reducing inpatient reimbursement for various conditions, including serious infections. The decision to treat IE on an outpatient basis is made more easily if based on previous published experience. Published reports have concluded that outpatient therapy is more appropriate for “uncomplicated” IE caused by relatively susceptible microorganisms. The purpose of this paper is to review data describing the results of management of IE in the outpatient setting.     

Gallbladder Infarction Complicating Infective Endocarditis

Infective endocarditis with systemic embolization of the gallbladder vasculature is very rare. We describe a case of subacute infective endocarditis in an adult complicated by embolization of the right hepatic artery with subsequent gallbladder infarction. In these cases, appropriate antibiotic therapy should be given before and after cholecystectomy. Gallbladder resection should be performed before valve replacement to decrease the potential seeding of a prosthetic valve.     

Pseudoaneurysm Formation in Infective Endocarditis

Left ventricular pseudoaneurysms that develop in the setting of infective endocarditis are believed to result from remodeling of extravalvular abscesses. The high pressure generated by the left ventricle is thought to dissect into the abscess causing it to form a characteristic sac‐like protuberance readily recognized echocardiographically. Left ventricular pseudoaneurysms most often arise from abscesses in the mitral‐aortic intervalvular fibrosa and protrude external to the aorta. Less often, as described herein, they arise from abscesses external the posterior mitral annulus and project into the posterior interventricular groove. Perforation may result in camo‐cameral or aorto‐cameral fistula formation, as well as fistulous communication with the pericardial space.     

Sinus of Valsalva Pseudoaneurysm as a Sequela to Infective Endocarditis

Pseudoaneurysm is an uncommon sequela of infective endocarditis. We treated a 44-year-old man who had an active case of group B streptococcal infective endocarditis of the aortic valve despite no evidence of valvular dysfunction or vegetation on his initial transesophageal echocardiogram. After completing 6 weeks of intravenous antibiotic therapy, the patient developed a sinus of Valsalva pseudoaneurysm and severe aortic regurgitation caused by partial detachment of the left coronary cusp.We used a pericardial patch to close the pseudoaneurysm and repair the coronary cusp. This case shows the importance of routine clinical follow-up evaluation in infective endocarditis, even after completion of antibiotic therapy. Late sequelae associated with infective endocarditis or its therapy include recurrent infection, heart failure caused by valvular dysfunction (albeit delayed), and antibiotic toxicity such as aminoglycoside-induced nephropathy and vestibular toxicity.     

Isolated Bacterial Meningitis as the Key Syndrome of Infective Endocarditis

Abstract.Background: Bacterial meningitis is an unusual first manifestation but a major complication of infective endocarditis.Patients and Methods: We present three well documented cases of isolated bacterial meningitis in endocarditis. Against this background we review the literature.Results: All patients presented with bacterial meningitis. Staphylococcus aureus was isolated in blood cultures of all patients, but was found only in the cerebrospinal fluid (CSF) of one patient. The underlying endocarditis was confirmed histologically in all three cases. Two patients recovered completely and one died.Conclusion: An extensive search for endocarditis is recommended in every case of an unusual isolate in bacterial meningitis whether it is isolated from blood or CSF.