Purulent Pericarditis in Sickle Cell Disease Due to Streptococcus agalactiae; a Unique Case Report and Literature Review.

Purulent pericarditis is a localized infection with a thick, fibrinous hypercellular exudate and is historically associated with a high mortality. We describe a case of purulent pericarditis due to Streptococcus agalactiae (S. agalactiae) in a 30-year-old woman with sickle cell disease who presented with fever, dyspnea, and S. agalactiae septicemia. Despite timely initiation of antibiotics, she developed a large purulent pericardial effusion requiring surgical pericardiocentesis followed by a pericardial window. At 14 months follow-up, she has remained asymptomatic without sequelae. A review of the literature contained only four patients with purulent pericarditis in sickle cell patients. We discuss the unique aspects of this case in the context of purulent pericarditis in the age of modern antibiotics and hypothesize on the pathogenesis of delayed pericardial effusion after pericarditis.

Cryoglobulinemia in a patient with chronic lymphocytic leukemia - A case report and review of literature of renal involvement in CLL.

The incidence of glomerulonephritis, as a manifestation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), has always been considered low. Though renal infiltration is usually detected at post-mortem, it does not often interfere with kidney function [1]. Though immunoglobulin (Ig) levels in most CLL patients are subnormal, small monoclonal Ig peaks are occasionally detected in serum. They were present in a number of reported CLL nephropathy patients, and not all were cryoglobulins; serum and glomerular staining were concordant for Ig type [2,3,4]. Myeloma, which secretes monoclonal light chains, causes nephropathy in 25% of patients. But the little presumably secreted by small plasma cell clones, without myeloma, may also be nephrotoxic. The same is true of the low secretory CLL cells, which may occasionally be associated with cryoglobulins and other nephrotoxic Igs [5]. We report a patient with early stage CLL (Rai stage 0) with cryoglobulins, which led to membranoproliferative glomerulonephritis (MPGN), and death. We located reports of 51 patients with CLL-associated nephrotic syndrome or nephropathy, mostly from MPGN related to local Ig deposits. In those patients screened for cryoglobulins, about half tested positive. Many were early stage cases, where MPGN developed long after CLL presentation, and responded to its treatment. As early diagnosis and treatment CLL-related nephropathy may be curative, we propose a prospective study to determine the incidence of hyperalbuminuria development after presentation.

Loss of Foxp3 is associated with CD30 expression in the anaplastic large cell subtype of adult T-cell leukemia/lymphoma (ATLL) in US/Caribbean patients: potential therapeutic implications for CD30 antibody-mediated therapy.

Adult T-cell leukemia/lymphoma (ATLL) is caused by the HTLV-1 virus, endemic to Japan and the Caribbean, and is likely derived from cells with the T-regulatory phenotype. The malignant cells express IL2 receptor α (CD25), and the majority express transcription factor Forkhead box P3 (Foxp3), in addition to T-cell markers. Occasional cases express CD30. Whereas Japanese cases are predominantly of the acute and chronic leukemic types, the less well-studied Caribbean cases are more often lymphomatous. We performed immunohistochemical analysis for CD25, Foxp3, and CD30 on samples from 42 US/Caribbean ATLL patients and correlated these markers with morphologic subtype and clinical characteristics. In the 16/42 patients who had successive biopsies, we determined the expression stability of these markers. Foxp3 was expressed in 26 of the 42 (62%) initial biopsies, and its intensity correlated with CD25 expression. It was more frequent in pleomorphic small-sized and medium-sized cell types than in large cell tumors but did not correlate with patients' clinical attributes. Foxp3 expression and morphology were unchanged in successive biopsies in 13 of 16 patients. Four initial biopsies had features of anaplastic large cell T lymphomas, all of which were Foxp3. Successive biopsies from 2 patients with pleomorphic medium cell variant showed diminishing expression of originally weak Foxp3 expression and de novo CD30 expression, whereas they showed morphologic progression to the anaplastic cell variant. A third patient's second biopsy revealed progression from pleomorphic medium to anaplastic large cell morphology with loss of Foxp3, but it remained CD30. Foxp3 expression correlates with pleomorphic small and medium cell types and may be lost with large cell transformation. The evolution of the latter type can be associated with the gain of CD30 expression; such ATLL tumors might respond to anti-CD30 monoclonal antibody therapies.

Attributes of brain metastases from breast and lung cancer.

BACKGROUND: Most brain metastases arise from breast and lung cancers. Few studies compare the brain regions they involve, their numbers and intrinsic attributes. METHODS: Records of all patients referred to Radiation Oncology for treatment of symptomatic brain metastases were obtained. Computed tomography (n = 56) or magnetic resonance imaging (n = 72) brain scans were reviewed. RESULTS: Data from 68 breast and 62 lung cancer patients were compared. Brain metastases presented earlier in the course of the lung than of the breast cancer patients (p = 0.001). There were more metastases in the cerebral hemispheres of the breast than of the lung cancer patients (p = 0.014). More breast than lung cancer patients had cerebellar metastases (p = 0.001). The number of cerebral hemisphere metastases and presence of cerebellar metastases were positively correlated (p = 0.001). The prevalence of at least one metastasis surrounded with >2 cm of edema was greater for the lung than for the breast patients (p = 0.019). The primary tumor type, rather than the scanning method, correlated with differences between these variables. CONCLUSIONS: Brain metastases from lung occur earlier, are more edematous, but fewer in number than those from breast cancers. Cerebellar brain metastases are more frequent in breast cancer.

Correlation between the proportion of breast volume involved by locally advanced tumors and invasion of the skin and posterior structures.

AIM: To evaluate any differences between the percentages of involved breast volume, pathologic attributes, and tumor marker expression of T3 and T4a-c tumors in locally advanced breast cancers (BC). METHODS: All patients with T3N > 0 and T4a-c BC without evidence of distant metastasis (M0), presenting to the Breast Clinic from 1980 to 2010, were examined to determine whether their BC's involved ≥ 50% of their breast volumes, defined by gross replacement of at least one hemisphere. Core needle biopsy or post-mastectomy specimens from tumors involving a known percent of breast volume were evaluated for: (1) pathological grades and lympho-vascular invasion (LVI); (2) hormone receptor (ER/PR) expression > 0; and (3) epidermoid growth factor 2 (her2) over-expression (3+) by immune-histochemical staining or fluorescent in situ hybridization. RESULTS: The data base included 98 patients with T3N> 0 M0 and 120 with T4a-c, any N disease, M0 disease. T3 tumor masses involved 50% or more of the breast in 23/98 (24%), and T4a-c tumors 65/120 (54%) (P < 0.001). Only 1% of T3 tumors and 23% of T4a-c tumors presented with total breast replacement. There were no significant differences between the pathological attributes and marker expression of the T3 and T4a-c tumors. CONCLUSION: These data suggest that erosion of the overlying skin or underlying chest wall by some BC may be due to neglect and delay, rather than inherent biological aggressiveness.

Fine needle aspiration (FNA) cytology for diagnosis of cervical lymphadenopathy.

Patients with cervical adenopathy suspicious for malignancy are often referred to the Otolaryngology Service for tissue diagnosis. Confirmation of nodal involvement by upper aero-digestive tract tumors (UADT) is best obtained by fine needle aspiration (FNA). Reported studies of FNA for lymphoma diagnosis have yielded conflicting results. Retrospective review of charts and pathology of 161 patients diagnosed with lymphomas yielded 53 patients with cervical adenopathy without apparent UADT. FNA's were performed on 28, and were repeated nine times, for a total of 37. Eleven had Hodgkin's disease and 17 other types of lymphomas. Seven of 37 specimens contained only blood; 15 contained lymphoid cells, nine of which were designated "reactive." Lymphoid cells designated as "atypical" or "suspicious for lymphoma" were found in 13 of the 37 aspirates. Two were diagnostic of lymphoma. Lymphoma was confirmed by histopathologic specimens in all patients, obtained 0-941 days (median 15, mean 73 days) after initial FNA. In lymphoma patients with cervical lymphadenopathy, FNA does not usually suffice for, and often leads to significant delays in diagnosis.

Rising incidence of tumorectomies without breast radiation in patients treated for invasive breast cancer in Caribbean nations.

BACKGROUND: Tumorectomy for invasive breast cancer (BC) is followed by local recurrence in 30% of patients who do not receive radiotherapy. In the United States 88% of tumorectomy patients receive radiation therapy. Many Caribbean nations lack radiation facilities and access to existing facilities is limited. METHODS: The charts of the 95 breast clinic patients treated in Caribbean nations for primary BC between 1980 and 2008 were reviewed. The nation of origin, original treatments, reported physician recommendations, and status at presentation to our clinic were recorded. RESULTS: Mastectomies (MCT) had been performed on 51 patients and tumorectomies (TCT) on 39. The ratio of TCT to MCT from 1980 to 1991 was 0.19, and then rose to 0.94, with a slight increase since. Only 6 of the 33 (18%) TCT patients had received radiation therapy. Patient accounts of why they had not been irradiated were available for 20 of 33: 12 denied referral for radiation, 4 refused it, and 4 had not been able to obtain it in their nation or region. At presentation to our clinic, 22 of the 51 MCT patients (43%) and 23 of the 33 TCT patients (70%) had locally recurrent BC. CONCLUSIONS: TCT entails significant risk of local relapse in nations without, or with insufficient radiotherapy facilities for their populations.

Localized nasal cavity, sinus, and massive bilateral orbital involvement by human T cell leukemia virus 1 adult T cell lymphoma, with epidermal hypertrophy due to mite infestation.

HTLV1 adult T cell lymphoma occurs tends to be widely disseminated and aggressive, with only brief responses to chemotherapy. Aside from cervical adenopathy, involvement of head and neck structures is uncommon and orbital involvement rare.We report a case of nasal cavity HTLV lymphoma with massive bilateral orbital involvement and proptosis, resulting in complete left and partial right eye amaurosis. No other sites of disease were found. Response to chemotherapy was rapid and complete, with almost complete restoration of vision and oculo-motor function; the patient has remained in remission for one year. An associated problem was striking bilateral hypertrophic, hyperkeratotic eyelid and breast lesions due to mite infestation.

Effective treatment of the brachial plexus syndrome in breast cancer patients by early detection and control of loco-regional metastases with radiation or systemic therapy.

BACKGROUND: In breast cancer (BC) patients the brachial plexus syndrome (BPS) has been reported to be due to loco-regional metastases or radiation plexopathy. Associated arm edema is considered more suggestive of the latter. Radiation therapy is the only effective treatment for BPS reported. METHODS: The charts of all BC patients who presented to our clinic from 1982 to 2006 with homolateral arm pain and neurological deficits, without humerus, cervical spine, or brain metastases, were reviewed. RESULTS: There were 28 patients fulfilling these criteria for BPS. Supraclavicular, axillary or chest wall metastases developed synchronously with the BPS in 26 patients; in 21 they were recurrences, found 6-94 months (median 34 months) after primary BC treatment, while in 5 others they were progressing inoperable primary tumors and nodes. Arm edema first occurred at the same time as loco-regional metastases in 19 patients. Treatment for the BPS was administered to 22 patients; it was directed at their locoregional metastases. The BPS was initially treated with radiation (8 patients) or chemo- or endocrine therapy (14 patients); 19 (86%) had partial or complete remission of pain and neurologic deficits, with an 8-month median duration. CONCLUSION: The BPS in BC patients is due to loco-regional metastases and is often associated with arm edema. Chemo- or endocrine therapy induced the remission of pain and deficits as frequently as radiation therapy.

Elevated red cell distribution width in the diagnosis of thrombotic thrombocytopenic purpura in patients presenting with anemia and thrombocytopenia.

OBJECTIVE: To determine if red cell distribution width (RDW) is elevated in thrombotic thrombocytopenic purpura (TTP) and to evaluate the sensitivity and specificity of such elevation in the diagnosis of TTP. METHODS: We retrospectively studied red cell distribution width at presentation in 25 consecutive patients with newly diagnosed TTP who were treated with plasmapheresis in our institution between 1997 to 2005. Control patients consisted of 20 consecutive patients without TTP who presented to the emergency room (ER) with anemia and thrombocytopenia. RESULTS: Patients with TTP had significantly elevated RDW compared with the controls (23.1% versus 17.2% respectively, P = 0.0001). An RDW > 18% had a 95% sensitivity and 70% specificity in the diagnosis of TTP, whereas RDW > 19% had 80% sensitivity and 85% specificity. CONCLUSIONS: TTP should be suspected in patients presenting with unexplained anemia and thrombocytopenia with an RDW > or = 18%.

Tapering and discontinuation of glucocorticoid prophylaxis during prolonged weekly to biweekly paclitaxel administration.

BACKGROUND: Taxanes cause hypersensitivity reactions, averted by premedication with H1 blockers and high glucocorticoid (GC) doses. Prolonged weekly taxane administration may lead to GC toxicity. PURPOSE: To determine whether patients not hypersensitive to initial paclitaxel (PTX) infusion after high-dose GC premedication will tolerate subsequent, prolonged PTX treatment without GC prophylaxis. PATIENTS AND METHODS: In 115/122 breast cancer patients not hypersensitive to initial PTX treatment, 20 mg dexamethasone (DXM) doses were tapered by 2.0 mg/week, reaching 0 in those receiving 9 or more courses. After 4 PTX courses, diphenhydramine was administered orally, rather than intravenously. RESULTS: PTX was administered 143 times after 2.0-5.0 mg of DXM and 357 times without DXM. A total of 46 patients received 1-40 PTX courses without DXM. None of these 115 patients experienced hypersensitivity reactions. CONCLUSION: Patients unreactive to their first PTX infusions, after high-dose and tapering GC premedication, may not require GC prophylaxis for subsequent PTX therapy.