Blastic plasmacytoid dendritic cell neoplasm: a rare external ear lesion presenting with leukaemia.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy, typically characterised by cutaneous lesions and bone marrow involvement. We present a unique case of a woman in her 70s, initially seen for a spontaneous swelling on her left external ear resembling a haematoma, which recurred after initial treatment, triggering further evaluation.Diagnostic challenges arose as the patient displayed positive markers for Myeloperoxidase (MPO) (p-ANCA), suggesting vasculitis. Dermatology considered various differential diagnoses, but imaging and tests ruled out significant pathology. Steroid treatment led to improvement, but coincided with a surge in white cell count (WCC), prompting an urgent haematological review.Subsequent investigations, including a punch biopsy of the external ear and a bone marrow biopsy revealed BPDCN concurrent with chronic myelomonocytic leukaemia. This case highlights the challenging diagnostic journey, emphasising the need for multidisciplinary collaboration and the potential for unique BPDCN presentations, expanding our understanding of this malignancy.

Extramedullary chronic phase chronic myeloid leukaemia (CML) involving the central nervous system: A case report.

Chronic myeloid leukaemia (CML) has been classically described as a disease restricted to the bone marrow with very few reports of extramedullary involvement. CNS involvement with CML has been described in the literature as an aggressive disease in the leukaemic phase either preceding or coexisting with medullary blast crisis or seen in patients with long-term Imatinib therapy. No treatment consensus exists for this patient group and outcomes remain poor. We hereby present a very rare report of CNS involvement with chronic phase CML at diagnosis in a patient who presented with raised intracranial pressure and cranial nerve palsies.

Real-world Outcomes With Rituximab-based Therapy for Posttransplant Lymphoproliferative Disease Arising After Solid Organ Transplant.

BACKGROUND: Optimal upfront therapy for posttransplant lymphoproliferative disease (PTLD) arising after solid organ transplant remains contentious. Rituximab monotherapy (R-Mono) in unselected patients has shown a lack of durable remissions. Cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP)-based chemotherapy confers improved response rates, although concerns exist about toxicity. METHODS: This multicenter retrospective study reports outcomes for adults with biopsy-proven B-cell PTLD treated initially with R-Mono or Rituximab plus CHOP (R-CHOP). Selection of therapy was made according to physician preference. RESULTS: Among 101 patients, 41 received R-Mono and 60 had R-CHOP. Most (93%) had undergone renal or liver transplantation. R-CHOP showed a trend toward improved complete (53% versus 71%; P = 0.066) and overall (75% versus 90%; P = 0.054) response rates. In the R-Mono group, 13 of 41 (32%) subsequently received chemotherapy, while 25 of 41 (61%) remained progression-free without further therapy. With median follow-up of 47 months, overall survival (OS) was similar for R-Mono and R-CHOP, with 3-year OS of 71% and 63%, respectively (P = 0.722). Non-PTLD mortality was 3 of 41 (7%) and 4 of 60 (7%) within 12 months of R-Mono or R-CHOP, respectively. The International Prognostic Index was statistically significant, with low- (0-2 points) and high-risk (≥3 points) groups exhibiting 3-year OS of 78% and 54%, respectively (P = 0.0003). In low-risk PTLD, outcomes were similar between therapies. However, in high-risk disease R-Mono conferred an inferior complete response rate (21% versus 68%; P = 0.006), albeit with no impact on survival. CONCLUSIONS: Our data support R-Mono as initial therapy for PTLD arising after renal or liver transplantation. However, upfront R-CHOP may benefit selected high-risk cases in whom rapid attainment of response is desirable.

Primary gut aspergillosis in a patient with acute myeloid leukaemia: the importance of early suspicion and definitive treatment.

Aspergillus infection is a known complication in immunocompromised patients, particularly in those with impaired neutrophil function. The pathophysiology of respiratory tract infection and disseminated disease are well understood, and guidelines exist for early detection and treatment. The gut has been speculated to be the potential portal of entry for Aspergillus, though previous case series outline that this is often discovered late and results in high morbidity and mortality. Early clinical suspicion, with definitive surgical intervention and antifungal treatment with voriconazole, can significantly increase the chances of survival. In this article, the authors discuss a case of primary gut aspergillosis with secondary dissemination in a patient with acute myeloid leukaemia who developed serious sequelae.

Are surrogate assumptions and use of diuretics associated with diagnosis and staging of acute kidney injury after cardiac surgery?

BACKGROUND AND OBJECTIVES: This study measured the association between the Acute Kidney Injury Network (AKIN) diagnostic and staging criteria and surrogates for baseline serum creatinine (SCr) and body weight, compared urine output (UO) with SCr criteria, and assessed the relationships between use of diuretics and calibration between criteria and prediction of outcomes. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This was a retrospective cohort study using prospective measurements of SCr, hourly UO, body weight, and drug administration records from 5701 patients admitted, after cardiac surgery, to a cardiac intensive care unit between 1995 and 2006. RESULTS: More patients (n=2424, 42.5%) met SCr diagnostic criteria with calculated SCr assuming a baseline estimated GFR of 75 ml/min per 1.73 m(2) than with known baseline SCr (n=1043, 18.3%). Fewer patients (n=484, 8.5%) met UO diagnostic criteria with assumed body weight (70 kg) than with known weight (n=624, 10.9%). Agreement between SCr and UO criteria was fair (κ=0.28; 95% confidence interval 0.25-0.31). UO diagnostic criteria were specific (0.95; 0.94-0.95) but insensitive (0.36; 0.33-0.39) compared with SCr. Intravenous diuretics were associated with higher probability of falling below the UO diagnostic threshold compared with SCr, higher 30-day mortality (relative risk, 2.27; 1.08-4.76), and the need for renal support (4.35; 1.82-10.4) compared with no diuretics. CONCLUSIONS: Common surrogates for baseline estimated GFR and body weight were associated with misclassification of AKIN stage. UO criteria were insensitive compared with SCr. Intravenous diuretic use further reduced agreement and confounded association between AKIN stage and 30-day mortality or need for renal support.

Radical renal surgery (laparoscopic and open) in octogenarians.

AIM: An ageing population is at significant risk of developing of renal cell carcinoma (RCC). We evaluate our units experience in managing RCC in octogenarians using either laparoscopic or open radical nephrectomy, highlighting the postoperative complication rates and survival outcomes. MATERIALS AND METHODS: From June 2001 to June 2008, 65 octogenarians underwent a radical nephrectomy for suspected renal cell carcinoma. The procedure was performed laparoscopically (group 1) in 29 patients (44%) and via an open nephrectomy (group 2) in 36 patients (56%). The presenting age, sex distribution, ASA score, preoperative co-morbidities and indications for nephrectomy were statically comparable in both groups. Postoperative complications were recorded using the Clavien-Dindo classification. RESULTS: Both groups were similar preoperatively with respect to age of presentation, ASA score and co-morbidities such as hypertension, ischemic heart disease, and chronic respiratory disease. Group 1 showed better statistically significant operative parameters (operative time and blood loss), mean length of hospital stay and most importantly postoperative complications. Postoperative complication rates were lower in group 1 (48.3%) when compared with group 2 (80.5%) (p<0.05). CONCLUSION: Surgery for renal cancer in patient over the age of 80 should only be considered after a thorough work up. Chronological age itself should not be the only determining factor. If such a surgery was to be undertaken, then in our experience, patients who underwent laparoscopic radial nephrectomy had fewer complications than those had open radical nephrectomy.