ABSTRACT
Here we described a case of an asymptomatic 73 years-old female patient in geriatric routine consultation, whose laboratory testing showed hyperproteinemia with accompanying hyperglobulinemia. A diagnosis of BGUS was made only after a correlation among SPEP, densitometry tracing and IFE results was established, evidencing a second peak, that was less evident and not reported at first. These biclonal conditions are of very low incidence in the clinical laboratory, requiring the laboratory professional to have particular skills for their identification. As far as is known, clinical findings in BGUS are similar to those found in MGUS. However, they remain not well understood. Therefore, for an accurate diagnosis of BGUS, the clinical laboratory technician must be trained and sensitized to detect a second M - protein as a band or peak; taking in mind the possible different scenarios in heavy and light chain typing.
Se describe el caso de paciente asintomática de 73 años de edad en consulta geriátrica de rutina, cuyos estudios de laboratorios muestran hiperproteinemia acompañada de hiperglobulinemia. Se estableció el diagnóstico de GBSI después de correlacionar entre resultados de electroforesis de proteínas, trazo de densitometría e inmunofijación en suero, los cuales evidenciaron un segundo pico monoclonal menos evidente y no reportado de primera instancia. Este tipo de condiciones biclonales son de muy baja incidencia en laboratorio clínico, lo cual requiere que profesional de laboratorio tenga ciertas habilidades para su identificación. Hasta donde se conoce, los hallazgos clínicos de GBSI son similares a aquellos encontrados en GMSI. Sin embargo, continúan sin ser bien comprendidas. Por tanto, a fin de un diagnóstico más preciso, el técnico de laboratorio debe estar entrenado y sensibilizado para encontrar una segunda proteína M como banda o pico, tomando en cuenta los diferentes posibles escenarios en la tipificación de cadenas pesadas y ligeras.
ABSTRACT
Mixed phenotype acute leukemia (MPAL) in adults represents nearly 2 to 5 % of all acute leukemia cases. There are two large studies throughout the world and only case reports and small series have been reported in Latin America. This study retrospectively analyses the clinical characteristics and survival of 27 patients with MPAL evaluated in three medical institutions of Mexico. All cases meet World Health Organization 2008 criteria; 70.3 % of patients had B lymphoid/myeloid lineage MPAL. Induction chemotherapy protocols included 7 + 3 hyper-CVAD, high-density schedules, and pediatric-like regimens such as New York II and total XI. Complete remission was achieved in 23/27 patients (85.2 %). Only one patient died due to chemotherapy-induced aplasia during remission induction (5.2 %). In 68 % of cases, we were able to administer maintenance therapy as a regimen in lymphoblastic leukemia. At the time of analysis, 70.4 % of the patients in the entire cohort had died mainly as result of disease progression (73.6 %). Disease-free survival was 13 months (95 % CI, 9.6-16.3 months) and overall survival was 14.8 months (95 % CI 13.4-16.27). Survival rates are low and standardized therapy for the management of this type of leukemia is still lacking. This is the largest series reported in Mexico and to the best of our knowledge in Latin America.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Immunophenotyping , Leukemia, Biphenotypic, Acute/diagnosis , Leukemia, Biphenotypic, Acute/drug therapy , Adolescent , Adult , Aged , Aged, 80 and over , Cohort Studies , Female , Humans , Leukemia, Biphenotypic, Acute/epidemiology , Male , Mexico/epidemiology , Middle Aged , Retrospective Studies , Survival Rate/trends , Treatment Outcome , Young AdultABSTRACT
Anaplastic lymphoma kinase-positive large B-cell lymphoma is a rare and aggressive B-cell lymphoma mostly associated with t(2:17) involving the clathrin gene at 17q23 and the anaplastic lymphoma kinase gene at 2p23. The characteristic immunophenotype includes a granular cytoplasmic anaplastic lymphoma kinase expression, CD20 negativity and the presence of plasma cell markers (CD138, VS38c, and CD38). We report a case with aberrant immunophenotype (CD138-, VS38c-, CD38+/-) and discuss the utility of other immunohistochemical markers in establishing a terminal B-cell differentiation.
