De-Novo B-Cell Prolymphocytic Leukemia.

B-cell prolymphocytic leukemia (B-PLL) is a rare B-cell neoplasm that typically presents with splenomegaly, a rising white blood cell count, and may or may not have B symptoms. The diagnosis usually requires a bone marrow biopsy and aspirate with flow cytometry and cytogenetic studies. At least 55% of the lymphocytes in the peripheral blood must be prolymphocytes to be defined as B-PLL. A thorough differential diagnosis would include mantle cell lymphoma, chronic lymphocytic leukemia (CLL) with prolymphocytes, hairy cell leukemia, and splenic marginal zone lymphoma. B-PLL is managed with regimens utilized for CLL, such as ibrutinib and rituximab but is tailored for each individual. The authors report a rare case of B-PLL in a patient with no known history of CLL. The authors discuss this entity in context of the 2017 and 2022 World Health Organization (WHO) classifications, the latter of which no longer recognizes B-PLL as a distinct entity. The authors hope that this article helps practitioners with the diagnosis and treatment of B-PLL. Perhaps with better recognition, and better documentation of histopathologic features of these rare cases going forward, it may prove to be a distinct entity again in future classifications.

Pathological Complete Response of Metastatic Clear Cell Renal Carcinoma with Pembrolizumab and Axitinib: A Case Report and Review of Literature.

The role of cytoreductive nephrectomy has become unclear since the introduction of immunotherapy which is now the backbone of the treatment for metastatic renal cell carcinoma. Different combinations are used based on the prognosis. Achieving a complete response would be ideal and includes radiographic disappearance of lesions. However, there have been a few reported cases of pathological complete response with persistent radiographic evidence of cancer. The authors report a case of pathological complete response despite persistent radiographic evidence of residual disease in a patient with metastatic renal cell carcinoma treated with pembrolizumab and axitinib. The patient subsequently underwent cytoreductive nephrectomy after the 13th dose of pembrolizumab. The resected mass consisted of scar tissue with no viable tumor cells seen on pathology but only scar tissue. This case reveals that persistent radiographic evidence of the tumor may be explained by scar tissue, challenging the role of cytoreductive nephrectomy in the era of immunotherapy.

Ten-year survival outcomes for patients with early stage classical Hodgkin lymphoma: An analysis from Kentucky Cancer Registry.

OBJECTIVE/BACKGROUND: Early stage classical Hodgkin lymphoma (cHL) has an excellent outcome. Recent studies focus on decreasing toxicity related to the addition of radiation along with chemotherapy. Real-life reporting of the addition of radiation to chemotherapy is lacking. This study investigates the outcomes obtained from a statewide cancer registry for early stage cHL patients treated with chemotherapy alone (CT) versus patients treated with the combined modality of chemotherapy and radiation (CMT). METHODS: A retrospective study of cHL patients diagnosed and treated was identified using a statewide cancer registry from 2005 to 2014. Patients with early stage disease (I, II) were then grouped on the basis of the presence of B symptoms into favorable and unfavorable groups. Baseline characteristics (age, gender, extranodal involvement, and histology) as well as overall survival were compared for both groups depending on whether they received CT or CMT as first line therapy for their cHL. RESULTS: A total of 961 patients were identified; of those, 127 were excluded as they received only radiation or another form of treatment. Of the remaining patients, 293 were categorized as early stage favorable cHL (Group 1) and 130 adults were in the unfavorable cHL (Group 2). There were 335 patients with advanced stage cHL (Group 3) and 76 patients in an unknown stage. The 10-year overall survival for Group 1 was 81.3% versus 76.3% for Group 2 and 52.7% for Group 3. For Group 1, 10-year overall survival was 86.7% with CMT versus 75.1% for those receiving CT only (p = .004). For Group 2, there was no difference in 10-year overall survival between the CMT group (80.0%) and CT (72.5%) (p = .73). CONCLUSION: While radiation therapy might increase long-term toxicity in cHL, in our large data cohort, radiotherapy consolidation as part of the initial therapy for early stage disease provides superior survival at 10 years, especially in favorable risk cHL.

Anti-thymocyte globulin in haematology: Recent developments.

Anti-thymocyte globulin (ATG) is a polyclonal antiserum introduced into clinical medicine more than 30 years ago. It induces a broad non-specific immunosuppression. In haematology, standard indications are severe aplastic anaemia and prophylaxis and treatment of graft-versus-host disease (GVHD) (after allogeneic transplantation). For aplastic anaemia, ATG from horses has been found to be superior to ATG from rabbits. In the situation of allogeneic transplantation, ATG lessens the risk of chronic GVHD but may not improve survival. There is current controversy regarding which patients benefit most from ATG and what the ideal dosage is. It is likely that in the coming years a more specific immunosuppressive will be developed that will minimize GVHD while maintaining the graft-versus-malignancy effect.

Irritable Bowel Syndrome and Bronchial Asthma: Are They Associated in Indian Population?

INTRODUCTION: Irritable Bowel Syndrome (IBS), a common gastrointestinal (GI) disorder has been linked with asthma implying a clue to its pathophysiology as being some form of allergic response. Previous studies from other parts of the world have shown both the presence as well as an absence of an association between them. AIM: We conducted a cross-sectional study to find an association between IBS and asthma, in an Indian cohort. MATERIALS AND METHODS: Seventy patients of IBS diagnosed by exclusion and ROME III criteria with a matched control group were questioned for GI, respiratory symptoms along with their spirometry analysis. Those having reversible bronchoconstriction were categorized as asthmatic. RESULTS: The prevalence of asthma in the IBS group of 16 females and 54 males was 12 (17.14%) as compared to 14 (20%) in control group. Chi-square test revealed the Odds Ratio (OR) for prevalence of asthma in IBS as 0.828 with 95% Confidence Interval (CI) between 0.320 and 2.121 (p=0.664) which was non-significant (p>0.05). CONCLUSION: This study denies the presence of association between IBS and asthma among Indian cohorts and suggests that regional variations are present in the above association.

Paraneoplastic pemphigus as a presentation of acute myeloid leukemia: Early diagnosis and remission.

Skin lesions are frequently encountered in clinical practice which can be a presentation of systemic diseases not excluding an occult malignancy. Commonly reported paraneoplastic dermatologic manifestations include acanthosis nigricans, dermatomyositis, erythroderma, hypertrophic osteoarthropathy, Sweet syndrome, and paraneoplastic pemphigus (PNP). PNP is a rare autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphic skin eruptions, and associated underlying neoplasms most commonly non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman disease. PNP is characterized on histopathology as dyskeratotic epithelial cells with acantholysis with a typical immunofluorescence staining pattern of direct and/or indirect staining of intercellular, basement membrane, and dermoepidermal junction with immunoglobulin-G and C3. PNP has been described to have poor prognosis with a mortality range of 75-90% and a mean survival of less than 1year. We describe a previously unreported case of PNP associated with acute myeloid leukemia (AML) where the patient presented with a nonhealing ulcer and hemorrhagic crusting on the face that did not respond to antimicrobials and steroids. Investigations revealed leukocytosis with peripherally circulating blasts. Skin biopsy revealed an evolving PNP and bone marrow biopsy confirmed evidence of AML. The patient underwent induction, consolidation, and then successful allogenic bone marrow transplantation with complete remission. The skin lesion, which was initially refractory to treatments, surprisingly resolved within 7days of starting induction chemotherapy. In this case, the skin lesion was a key factor in early diagnosis and instituting treatment for the underlying AML. Early intervention gave our patient a better outcome with an ongoing survival of 18months since diagnosis, maintaining complete remission.

Impact of a venous thromboembolism prophylaxis "smart order set": Improved compliance, fewer events.

Venous thromboembolism (VTE) affects over 700,000 Americans annually. Prophylaxis reduces the risk of VTE by 60% but many patients still do not receive risk-appropriate VTE prophylaxis. To improve our institution's VTE prophylaxis performance, we developed mandatory computerized clinical decision support-enabled "smart order sets" that required providers to assess VTE risk factors and contraindications to pharmacologic prophylaxis. Using provider responses, the order set recommends evidence-based risk-appropriate VTE prophylaxis. To study the impact of our "smart order set" on prescription of risk-appropriate VTE prophylaxis and clinical outcomes, we conducted a retrospective chart review of consecutive patients admitted to the Medicine service during one month immediately prior to (November 2007) and a single month subsequent to (April 2010) order set launch. Data collection included patient demographics, VTE risk factors, and the use and type of VTE prophylaxis. The pre- and post-implementation cohorts contained 1,000 and 942 patients, respectively. After implementation of the "smart order set", the prescription of risk-appropriate VTE prophylaxis increased from 65.6% to 90.1% (P < 0.0001). Orders for any form of VTE prophylaxis increased from 76.4% to 95.6% (P < 0.0001). Radiographically documented symptomatic VTE within 90 days of hospital discharge declined from 2.5% to 0.7% (P = 0.002). Preventable harm was completely eliminated (1.1% to 0%, P = 0.001) with no difference in major bleeding or all-cause mortality. A VTE prophylaxis computerized clinical decision support-enabled "smart order set" improved prescription of risk-appropriate VTE prophylaxis, reduced symptomatic VTE and eliminated preventable harm from VTE without increasing major bleeding.