Sanju Cyriac

Nonadherence to Imatinib adversely affects event free survival in chronic phase chronic myeloid leukemia

There is limited data on the impact of treatment interruptions due to nonadherence in patients with chronic phase chronic myeloid leukemia (CP‐CML) treated with Imatinib. We looked at factors (including adherence to therapy) affecting the outcome in a large cohort of patients with CP‐CML. All the 516 patients received Imatinib free‐of‐cost through a company sponsored scheme, which mandated regular three monthly visits for drug procurement. Data regarding the disease characteristics, adherence to treatment and outcomes, were obtained from patients records. Unwarranted interruption of treatment for more than 1 week was defined as nonadherence. With a median follow‐up of 39 months, the estimated 5‐year event free survival (EFS) was 70.8% (95%, CI = 63.3–78.3). Nearly one‐third of the patients (29.6%) were found to be nonadherent at some point during their treatment. On univariate analysis, the factors adversely affecting the EFS were prolonged symptom duration before diagnosis, treatment with hydroxyurea for more than 1 month before start of Imatinib, and nonadherence to therapy. Only nonadherence was significant in multivariate analysis (HR1.6; P = 0.048). The 5‐year EFS in adherent and nonadherent patients was 76.7% and 59.8% respectively (P = 0.011, log rank test). Nonadherent patients were less likely to achieve complete cytogenetic responses (26% versus 44%; P = 0.004; χ2 test) at any point. A significant proportion of patients with CP‐CML have drug interruptions due to nonadherence during therapy and this compromises the EFS. Adherence to therapy must be included as an important evaluation parameter in all future studies of CML. Am. J. Hematol. 2011.

Etoposide, cisplatin–etoposide, methotrexate, actinomycin-D as primary treatment for management of very-high-risk gestational trophoblastic neoplasia

To evaluate the efficacy of etoposide, cisplatin–etoposide, methotrexate, actinomycin‐D (EP–EMA) chemotherapy as the frontline treatment for gestational trophoblastic neoplasia (GTN) patients with very high (≥ 12) FIGO prognostic scores.

Immune hemolytic anemia in a patient with tuberculous lymphadenitis.

Anemia in tuberculosis is usually anemia of chronic disease. Severe hemolytic anemia is exceedingly rare in tuberculosis patients. We report a patient diagnosed with tubercular lymphadenitis complicated by Coombs positive hemolytic anemia. Patient responded well to antituberculous treatment. Hematological parameters improved after initiation of antituberculosis treatment. To the best of our knowledge, this is the first case from India of an adult patient with tuberculous lymphadenitis presenting with Coombs positive hemolytic anemia.

Hypereosinophilia in hodgkin lymphoma

The incidence of eosinophilia in Hodgkin lymphoma is approximately 15%. Both peripheral and tissue eosinophilia have been noted in Hodgkin lymphoma. Eosinophils have important role in pathobiology of Hodgkin lymphoma. The mechanism of eosinophilia remains unknown though various mediators like IL-5 and GM-CSF have been implicated. We present a case who was diagnosed to have Hodgkin lymphoma and hypereosinophilia.

T lymphoblastic lymphoma relapsing in skin — a rare clinical scenario

Prasad P. G. ( ) E-mail: drsanpan80@yahoo.com Master X was diagnosed to have T cell lymphoblastic lymphoma, St Judes stage III when he was 5-year-old in 2003. He was treated with MCP 841 protocol. He was in clinical remission and was on follow up. He presented with a painless noduloulcerative purplish skin lesion 3 × 4 cm (Fig. 1) on anterior abdominal wall with slough of 3 months duration. Pallor, lymphadenopathy and hepatosplenomegaly were absent. Testes were of normal size and nervous system examination was normal. He had normal hemogram including differential count. Bone marrow studies including bilateral biopsies were normal. CT chest and abdomen were normal. Skin biopsy was done, which after immunohistochemistry was suggestive of non-Hodgkin lymphoma (NHL), high grade, blastic T cell immunophenotype (LCA, CD3 & Tdt +) (Fig. 1). Lymphoblastic lymphomas are predominantly tumors of childhood forming 6% of all pediatric NHL [1]. Eighty percent of the cases are T cell phenotype which classically presents with lymphadenopathy and mediastinal widening. Extranodal presentations are more common with B cell phenotype. The case presented above is unique in many ways. Though relapses can happen after intensive therapy, late relapse (>3 years) is uncommon. This reinstates importance of prolonged follow-up. The presented case has an isolated cutaneous relapse. Only few cases have been reported in world literature. Cutaneous lesions have been more frequently described with B cell lymphoblastic lymphoma. Ulceration is distinctively uncommon. Millot, et al. [2] studied the cutaneous involvement in children with acute lymphoblastic leukemia (ALL) or lymphoblastic lymphoma at diagnosis. They found cutaneous involvement is most common on the head and mainly with B cell precursor phenotype. Balduzzi, et al. [3] reported a case of T-ALL who relapsed while on maintenance therapy

Composite Hodgkin lymphoma and chronic lymphocytic leukemia: A rare case

Sir, Composite lymphomas (CL) are rare. They are increasingly been reported due to advances in immunohistochemical methods. The mechanisms described for the occurrence of composite lymphomas include clonal selection, genomic instability, immunosuppression, and congenital predisposition. The combination of Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL) is very rare. Sometimes, patients with typical chronic lymphocytic leukemia (CLL) may go on to develop HL, the so-called HL variant of Richter’s syndrome.

Malignant ectomesenchymoma of the nasal cavity

Malignant ectomesenchymomas are rare tumors. This tumor affects predominantly young children. Most common site is head and neck. A multi modality approach should be appropriate for this soft-tissue tumor. We present a 43 year old female with malignant ectomesenchymoma of the nasal cavity.

Early CNS toxicity after intrathecal methotrexate

Central nervous system (CNS) being a sanctuary site, intrathecal methotrexate (IT MTX) has become a cornerstone for CNS prophylaxis for acute lymphoblastic leukemia. Recently we noticed eight pediatric patients developing transient neurological event following IT MTX. All of them were acute lymphoblastic leukemia (ALL) patients. Four were high risk patients and others were standard risk (based on total count and age). All of them received BFM 86 protocol. Seven were females. Their age ranged from 4–14 years and the median age was 11.5 years. All the patients had normal CSF studies. Five of them received IT MTX along with intravenous high dose MTX (HDMTX) 5 gm/m before the event. Two received cytosine arabinoside (AraC), (75 mg/m for 4 days) with IT MTX on day one. Only one received cranial irradiation before the event. All the patients developed the event between Day 11 to D14 of IT MTX. Seven patients had hemiparesis and one had quadriparesis. The neurological event recovered within 24 hours in seven of them and within 72 hours on the others. Cerebrospinal fl uid (CSF) study was normal in all patients. All of them were exposed to multiple doses of IT MTX prior to the event. Preservative free MTX recommended dosage (12 mg) was used for all patients and proper administration technique under sterile precautions was ensured. Imaging studies were conducted in all patients within 24 hours of the onset of the event and all had normal standard MR imaging study of the brain. The imaging studies were not repeated later. The incidence of various neurological events following IT-MTX is estimated around 4–15% [1]. Three distinct clinical patterns of neurotoxicity have been observed (a) an acute toxicity, occurring within hours of IT chemotherapy, probably resulting from chemical arachnoiditis; (b) sub acute toxicity occurring within days or weeks, and characterized by symptoms of seizures, transient paresis, or cerebellar abnormalities; and (c) a delayed leukoencephalopathy form that is more commonly observed when IT MTX is used with cranial irradiation [1]. The mechanism of methotrexate induced neurotoxicity is poorly understood. Both high-dose intravenous MTX and intrathecal MTX are proposed to have association with demyelination, white matter necrosis, loss of oligodendroglia, axonal swelling, microcystic encephalomalacia, and atrophy relatively selective for the deep cerebral white matter [2]. An altered myelin metabolism disturbance induced by S. Cyriac · T. G. Sagar · R. Rajendranath Department of Medical Oncology, Cancer Institute, 18 Sardar Patel Road, Guindy, Chennai 600 036, India

L-asparaginase induced fatal cortical venous thrombosis in acute lymphoblastic leukemia

L-asparaginase has become an integral part in the treatment of acute lymphoblastic leukemia. The major worry of using L-asparaginase is thromboembolism. The case presented here is a 21-year-old lady who developed fatal cortical venous thrombosis during induction phase of treatment for ALL. Early recognition is very important to treat this potentially catastrophic yet treatable complication.