A 79-year-old woman with a history of hysterectomy and cholecystectomy.
In May 2010, the patient consulted due to fatigue, weight loss with intake, night sweats and spontaneous ecchymosis, which had been preserved for a year.
Upon physical examination, the patient had mental retardation, a mass located in the pelvis and a poorly defined hypogastric mass.
No lymphadenopathies were detected.
There was anemia, thrombocytopenia and lymphocytosis (some of a "wake" appearance).
She was hospitalized with presumptive diagnosis of hairy cell leukemia (tricoleukemia).
A scan showed giant scleromegaly (286 mm in length), adenopathies in the hepatic hilium and in the bottom of the douglas sac.
Myelogram showed hyperplasic erythropoietic hyperplasia, granulocytic hypoplasia, mastocytosis (5-7%), absence of erythropoietic lymphocytes (78%) and absent hemosiderin.
A bone marrow biopsy revealed hyperplasia of the three series and small lymphocytes CD20(+) and CD3(-).
Immunophenotype was not performed because the patient could not solve its cost.
The pathologist concluded that there was a B-cell lymphoproliferative syndrome.
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Other assessments showed elevated TSH (10.4 U.I./ml), coagulation disorders such as TTPK and prolonged prothrombin time that did not correlate with the addition of reduced IgM and anticardiolipin antibodies positive; factor VIII.
The concentration of immunoglobulin M was 7 times normal (monoclonal lambda immunofixation in blood) and beta 2 microglobulin was elevated.
Autoimmunity tests were negative except for rheumatoid factor.
The lesion was excised after transfusion of erythrocytes and platelets. A total of 2,533 grams were obtained. Biopsy revealed marked hypercellularity of red pulp, lymphoid tissue of atrophic white pulp.
90% of lymphoid cells were CD20(+), 10% CD3(+) and 50%BCL2(+).
It was concluded that the findings were compatible with a triucemia.
She was discharged with prescription of thyroxine and allopurinol.
The patient did not return to control.
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Twelve months later the patient developed dyspnea and nausea.
The examination revealed marked weakness, tachycardia and polypnea.
Blood pressure was 120/70 mmHg, there was no fever or edema.
The chest X-ray showed no abnormalities that could explain the dyspnea.
Blood glucose was normal and there was metabolic acidosis with elevated anion gap and lactate without evidence of hypoxemia, tissue hypoperfusion or systemic infection.
Urine examination was normal, and blood and urine cultures were negative.
Cardiac enzymes were not compatible with myocardial infarction and except for mild hyperbilirubinemia, liver tests showed no evidence of cytolysis or cholestasis.
Blood count revealed pancit, macrocytosis (MCV 108u3), Howell Jolly bodies and no "vellued lymphocytes" were visualized.
In this second hospitalization the concentration of IgM (monoclonal) was high 10 times the normal, IgA and IgG were reduced and there was an increase in uricemia, LDH and B2 microglobulin.
The echotomography showed heterogeneous hepatomegaly with steatosis and retroperitoneal lymphadenopathy.
The scanner showed a slightly enlarged conserved liver, normal kidneys, liver hilium adenopathies and Douglas sac fundus.
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With the diagnosis of ACLB associated with lymphoproliferative syndrome, allopurinol, corticosteroids and intravenous sodium bicarbonate were indicated, achieving to reduce acidosis but without significantly reducing lactate concentrations.
A spinal tap obtained a sample that was analyzed with flow cytometry.
2% were B-cell lymphoid cells with expression of CD45,CD19,CD20,CD11c,CD25 antigens and absence of CD10 and CD103.
The cells had restriction in light chains with expression of lambda chains.
It was concluded that there was a marginal lymphoma of the blaze, discarding the previous clinical diagnosis of triucemia.
Twelve days after admission, it was decided to administer an anti-CD20 antibody (rituximab) in order to reduce the mass of B lymphocytes of the patient.
Rituximab 281 mg (188 mg/m2) was administered as a progressive reduction in lactate levels until normalization and a rapid increase in platelet count.
Subsequently, the usual dose (375 mg/m2) was administered within 3 weeks.
Normal lactate concentrations and IgM concentration decreased to 25% of baseline.
