J.P. Jouet

Three cases of translocation (8;16)(p11;p13) observed in acute myelomonocytic leukemia: A new specific subgroup?

In three cases of acute nonlymphocytic leukemia we observed a translocation (8;16)(p11;p13); in one case it was the sole karyotypic change and in the other two cases it was associated with other structural anomalies. All three cases were nonhyperleukocytic myelomonocytic leukemias with erythrophagocytosis by some blast cells and cytochemistry results consistent with leukemic proliferation of a common monocytic-granulocytic precursor. The importance of this translocation is discussed, and the implication of band 16p13 in myelomonocytic leukemia is stressed.

Promyelocytic blast crisis of Philadelphia-positive thrombocythemia with translocations (9;22) and (15;17).

We report a promyelocytic blast crisis in a case of Ph-positive thrombocythemia with both t(9;22) and t(15;17). Our patient confirms the specificity of t(15;17) in malignant proliferation of promyelocytes and suggests its appearance as a second genetic event in the genesis of blast crisis occurring in a Ph-positive clone.

Good Predictive Value of Combined Cytogenetic and Molecular Follow Up in Chronic Myelogenous Leukemia After Non T-cell Depleted Allogeneic Bone Marrow Transplantation: A Report on 38 Consecutive Cases

We prospectively performed repeated cytogenetic and PCR monitoring of residual disease in all cases Ph positive of chronic myeloid leukemia (CML) allografted with non T cell depleted marrow at our institution over a period of 8 years. Thirty eight patients who survived the immediate post transplant period could be analyzed (median of 3 cytogenetic analyses/patient, examining 100 mitoses, and 4 PCR analyses/patient). Seven of the 38 patients had a hematological relapse (which was extramedullary in one case) and one a purely cytogenetic relapse, possibly stabilised by interferon treatment. Within 6 months of transplant, Ph positive mitoses were seen in 2 patients, and positive PCR in most cases, without implying subsequent relapse. Six of the 32 patients analyzed cytogenetically more than 6 months post transplant had Ph positive mitoses on at least one occasion: 5 had a hematological relapse within 7 months of positive cytogenetic analysis, and the remaining patient, treated by interferon remained in purely cytogenetic relapse. (The extramedullary relapse was not preceded by a positive marrow karyotype). Eight of the 38 patients had positive PCR findings on at least one occasion more than 6 months post transplant. Seven relapsed (6 hematological relapses including the extramedullary relapse, and 1 cytogenetic relapse) after 3 to 20 months, but the remaining patient remained in CR 36 months later, with negative PCR. The 30 patients who never had positive PCR findings remained in CR. In this relatively large series of patients, we found a good correlation between PCR findings more than 6 months post transplant and remission or relapse status.(ABSTRACT TRUNCATED AT 250 WORDS)

Pseudomembranous conjunctivitis following bone marrow transplantation: Immunopathological and ultrastructural study of one case

Pseudomembranous conjunctivitis (PMC) is a rare ophthalmic lesion usually caused by infectious agents such as streptococcus, Klebsiella, Chlamydia, or adenovirus. It can also be associated with skin lesions of toxic epidermal necrolysis (TEN) and graft-versus-host-disease (GVHD). We report the pathological study of a patient with PMC and GVHD who had a conjunctival biopsy before death. This allowed us to differentiate this form of PMC from PMC of infectious origin, and to find within the epithelium characteristic features of acute GVHD and mononuclear cells expressing natural-killer markers. This suggests that this form of PMC resulted from a very acute, cytotoxic form of GVHD. Because PMC in bone marrow recipients can be related to infections, TEN, or acute GVHD, a systematic pathological study is required to guide the therapy.

Acute Flare-up of Conjunctival Graft-versus-host Disease with Eosinophil Infiltration in a Patient with Chronic Graft-versus-host Disease

Chronic graft-versus-host disease (cGvHD) is a disabling alloimmune disease. Acute flare-ups of alloimmune reactions can occur even at the chronic clinical stage necessitating modulation of immunosuppression therapy. We studied conjunctival lesions of a patient with an acute flare-up in cGvHD. Along with severe GvHD lesions, biopsies showed eosinophils with pathological signs of activation. Since eosinophil recruitment and activation is observed in flare-ups of patients with autoimmune or inflammatory bowel diseases, we suggest that activated eosinophils in target organs may be a marker of evolutive lesions in alloimmune reactions as in other kinds of inflammatory diseases.

Translocation t(1;19)(q23;p13) in acute lymphoblastic leukemia: A report on six new cases and an unusual t(17;19)(q11;q13), with special reference to prognostic factors

We report clinical, immunologic, and cytogenetic characteristics of six patients with a t(1;19)(q23;p13) that was balanced in one case and of the unbalanced type [-19,der(19)t(1;19)(q23;p13)] in the remaining five cases. Intracytoplasmic immunoglobulins (cIg) were positive in the three cases where they were found. We also report on another patient, with a t(17;19) involving 17q11 and probably 19q13 regions, although involvement of 19p13 could not be excluded. In this patient, cIg were also present, thus raising the issue of whether such a rearrangement could be a variant of t(1;19). Clinically, five patients belonged to the high-risk acute lymphoblastic leukemia (ALL) group, because of high leukocytosis, central nervous system (CNS) disease at presentation, or massive organomegaly. Cytologically, all cases were FAB type L1. Except for the two cases allografted in the first complete remission (CR) all patients relapsed, three of them within 13 months. Two CNS relapses were seen in spite of adequate CNS prophylaxis. ALL with t(1;19) appears to be a poor-risk ALL subgroup and probably requires a reinforcement of therapeutic modalities that might include, when possible, allografting at first CR.

Un diagnostic différentiel de thrombocythémie essentielle à ne pas méconnaître: l'asplénie congénitale, une observation

We report a case of congenital asplenia in an adult revealed by isolated thrombocytosis. The biological features might mimic a primary thrombocythemia.

Les lymphomes à localisation testiculaire : analyse de 12 observations

Non-Hodgkins lymphomas with testicular involvement are unfrequent. Most cases occur in elderly subjects and are of intermediate or high grade malignancy. Our study suggests that an early intensive chemotherapy improves survival. Lymphoblastic or Burkitts lymphomas have a poor outcome and CNS relapses are frequent.