tretinoin and Osteolysis

Granulocytic sarcoma (GS) is a localized tumor composed of immature myeloid cells. This extramedullary tumor can present before, concurrent with or after the diagnosis of acute myeloid leukemia. GS is extremely uncommon in acute promyelocytic leukemia (APL). As a proportion of patients never develop systemic disease, correct and timely diagnosis may be rather difficult, but is a prerequisite for optimal outcome. GS should be considered in the differential diagnosis of children with unusual bone lesions. We describe a patient with GS who presented with symptoms mimicking osteomyelytis or rheumatoid disease.

Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Arthritis, Psoriatic; Biomarkers, Tumor; Diagnostic Errors; Female; Humans; Leukemia, Promyelocytic, Acute; Oncogene Proteins, Fusion; Osteolysis; Osteomyelitis; Remission Induction; Sarcoma, Myeloid; Shoulder Pain; Tretinoin

The association of leukemia and multiple myeloma is well described usually as a complication of chemotherapy but also in the absence of chemotherapy or at diagnosis. Such leukemias are typically acute myeloid leukemia (AML), particularly myelomonocytic subtype, and cases of acute promyelocytic leuke (APL) are rarely reported. Controversy exists as to whether myeloma and AML originate from a single haematopoietic progenitor or arise from different cell lineages. We report a case of a 58 year old female who developed APL 10 months following diagnosis of nonsecretory light chain (kappa) myeloma which had been treated with local spinal irradiation and low dose oral melphalan and prednisone. Clonality had originally been demonstrated by light chain restriction (kappa) of her bone marrow plasma cells whilst immunoglobulin heavy chain and T cell receptor genes were germ line. At development of APL cytogenetics revealed t(15;17) and PML-RAR fusion gene was detected by RT-PCR. The patient was treated with all-trans retinoic acid (ATRA) and received 2 cycles of consolidation chemotherapy with Idarubicin. Following this therapy the t(15;17) and PML-RAR were both undetectable whilst the clonal population of kappa staining plasma cells persisted. This particular patient represents a rare case of APL complicating multiple myeloma with persistence of the myeloma clone but disappearance of PML-RAR alpha RNA following therapy. This case study appears to support the argument that the APL and myeloma originated from distinct cell lineages.

Topics: Antibiotics, Antineoplastic; Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Cell Lineage; Chromosomes, Human, Pair 15; Chromosomes, Human, Pair 17; Clone Cells; Combined Modality Therapy; Diphosphonates; Embryonal Carcinoma Stem Cells; Female; Gene Rearrangement, B-Lymphocyte, Light Chain; Humans; Idarubicin; Immunoglobulin kappa-Chains; Leukemia, Promyelocytic, Acute; Melphalan; Middle Aged; Multiple Myeloma; Myeloma Proteins; Neoplasm Proteins; Neoplasms, Multiple Primary; Neoplastic Stem Cells; Oncogene Proteins, Fusion; Osteolysis; Pamidronate; Prednisone; Remission Induction; Translocation, Genetic; Tretinoin