Uncommon Haematological Transformation: Chronic Myeloid Leukaemia Transitioning Into Plasma Cell Leukaemia in a Single Patient.

A 40-year-old male patient who was a known case of chronic myeloid leukaemia (CML) was diagnosed two years back on tab imatinib 400 mg/day; he came with complaints of easy fatigability, syncopal attacks, and bone pain associated with low-grade fever for 15 days. Repeat haematological profile and a peripheral smear of the patient suggested features of plasma cell leukaemia (PCL)/plasma cell dyscrasia (PCD). A definitive treatment protocol of lenalidomide, bortezomib, and dexamethasone for PCL was prescribed to the patient. This medical case study emphasizes the rare possibility of the transformation of CML into PCL and the possible trigger of tyrosine kinase inhibitor for the same.

Chronic Myeloid Leukemia: Chronic Phase Presenting as Extramedullary Presentation.

Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by immature granulocytes in the peripheral blood and bone marrow. In 95% of cases, it is always associated with the presence of the Philadelphia chromosome, which is characterized by the presence of reciprocal translocation between chromosomes 9 and 22. However, 3.1-9.1% of patients also have an extramedullary proliferation of skin, lymph nodes, bone, or central nervous system (CNS), which could be either myeloid, lymphocytic, or mixed lineage in origin. An extramedullary myelogenous neoplasm termed myeloid sarcoma (MS) can originate from myeloblasts or immature myeloid cells. Due to the green, gross appearance caused by the myeloperoxidase enzyme in immature myeloid cells, it is also known as chloroma. According to WHO guidelines, it is a tumor composed of myeloid blasts, mature or immature. Here we report an old female patient with CML - chronic phase who came for imatinib therapy and presented as MS in the right parotid gland.

Novel formulation of cytarabine and daunorubicin: A new hope in AML treatment.

Acute myeloid leukemia (AML) treatment has always been a challenge to the treating physician. Continuous efforts are being made to improve treatment outcomes in AML. CPX-351 is a pharmacologic advancement in this direction. It is a liposomal fixed drug combination of cytarabine and daunorubicin. Early studies indicate that it will play a big role in AML treatment. This is a short review about this drug.

Management of relapsed-refractory diffuse large B cell lymphoma.

Relapsed-Refractory Diffuse Large B Cell Lymphoma (RR DLBCL), which accounts for approximately one-third of patients with DLBCL, remains a major cause of morbidity and mortality. Managing RR DLBCL continues to be a challenge to the treating hemato-oncologist. Salvage high-dose chemotherapy followed by autologous stem cell transplantation is the standard of care for chemosensitive relapses in DLBCL. Various salvage regimens are available, but the quest for an optimal regimen continues. The addition of rituximab to the salvage regimen has improved the outcome of RR DLBCL. Several pertinent issues regarding the management of RR DLBCL are discussed in this short review.

Homozygosity for the severe ß(+)-thalassemia mutation [IVS-I-5 (G>C)] causes the phenotype of thalassemia trait: an extremely rare presentation.

The thalassemias are the most common single gene disorder known to mankind. The phenotype of thalassemia depends upon the underlying gene defect in addition to many modulating factors. As the literature describes, inheritance of a ß(0) genotype in the homozygous state results in the development of ß-thalassemia (ß-thal) major with key clinical features being transfusion dependency, physical abnormalities and iron overload. IVS-1-5 (G>C) is the severe ß(+) allele whose homozygosity results in severe ß-thal. We describe a patient who was asymptomatic until screened and was found to have mild anemia. Detailed analysis revealed the presence of the IVS-I-5 mutation in a homozygous state that was unlikely to present as a transfusion independent state. The study of such cases emphasizes the complexity of genetic interactions that underlie the phenotype of ß-thal and highlight the importance of the regulation of Hb F production in ß-thal syndromes. Simultaneous inheritance of some loci that modulate Hb F levels probably causes high levels of total hemoglobin (Hb) and to be transfusion independent.

Chronic myeloid leukemia in children and adolescents: A single center experience from Eastern India.

CONTEXT: Chronic Myeloid Leukemia (CML) constitutes around 3% of leukemia in the children and adolescent age group. AIMS: The aim of the study was to evaluate the characteristics at presentation and the treatment outcome of CML in the children and adolescent age group. SETTINGS AND DESIGN: Retrospective analysis was carried out at a single center in India. MATERIALS AND METHODS: Thirteen patients (≤17 years) attending CML outdoor from April 2008 to August 2012 were included in the analysis. STATISTICAL ANALYSIS USED: The mean and median of various parameters were calculated using a Microsoft excel sheet. SPSS 16.0 version software was used to calculate OS and PFS. RESULTS: CML-CP was the most common phase at presentation. Maximum patients belonged to the 14 - 17 year old age group. Disease was common in the male sex. Splenic discomfort and asthenia were the most common symptoms and splenomegaly was the most common sign. CONCLUSIONS: The treatment with Imatinib was effective and well-tolerated.