Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The prognostic significance of immunophenotypical properties of leukaemic cells is well known. However, the biological and clinical significance of CD7 and CD56 antigen expression in acute leukaemias are not clearly established. In patients with acute leukaemias, we identified CD7 and CD56 expression and analysed their associations with markers expressed early in haemopoietic ontogeny and clinical parameters. Among 22 patients with acute leukaemia [12 acute myeloblastic leukaemia (AML), 10 acute lymphoblastic leukaemia (ALL)], we found CD7 positivity in 15 of 22 patients (68%) and CD56 positivity in four patients (18%). CD7 positivity was observed in seven patients (58%) with AML and in eight patients (80%) with ALL. CD56 positivity was observed in three patients (25%) with AML and one patient (10%) with ALL. Lymphadenopathy was present in five patients and associated with hepatosplenomegaly in three patients with ALL. Splenomegaly and hepatomegaly were present in three patients with AML. Central nervous system involvement was seen in one patient with ALL. Complete remission was achieved in nine patients (41%) (five ALL and four AML). Our data showed that CD7 and CD56 positivity at diagnosis associated with low remission rate and biological aggressiveness in a significant proportion of patients. We suggest the evaluation of CD7 and CD56 in all patients with acute leukaemias at the time of diagnosis in view of poor clinical outcome.
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PMID:The importance of CD7 and CD56 antigens in acute leukaemias. 1505 63

BACKGROUND Most patients with chronic lymphocytic leukemia (CLL) are asymptomatic at diagnosis, but 10% present with B symptoms. Most patients have palpable lymphadenopathy, while 20-50% of the patients have hepatosplenomegaly. Cutaneous infiltrations in patients with CLL can be localized or generalized in the form of erythematous papules, plaques, nodules and, ulceration, which is uncommon. CASE REPORT We present the case of a 71-year-old man diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with atypical immunophenotype and increased prolymphocytes (CLL/PLL), which was treated initially after white blood counts (WBC) doubling with Bendamustine and Rituximab for 6 cycles, and achieved complete remission. The patient relapsed after 6 months of completion of treatment, with multiple large subcutaneous masses, proved to be infiltration with the same atypical CLL/SLL on tissue biopsy, with pathologic features indicating disease progression. The lack of similar reported cases, and the aggressiveness of the tumor clinically and histopathologically, resulted in the decision to treat with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone (R-CHOP) as a case of aggressive lymphoma, with complete remission clinically and radiologically. CONCLUSIONS We present a rare case of subcutaneous extramedullary masses of atypical CLL/SLL. The high proliferation index (Ki-67) and the increase of large cells are suggestive of aggressive progression of the disease; however, no frank features of Richter's transformation were noted. Based on this and because of the unusual aggressive-looking skin masses, the panel decided to treat the patient with R-CHOP. The impact of this presentation on the prognosis of the disease is not clear. To date, our patient has responded well to treatment with R-CHOP, with complete remission of the subcutaneous masses and on PET scan, but further follow-up is needed.
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PMID:Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge. 3238 31