Subject(s)
Fusion Proteins, bcr-abl , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Thrombocytopenia/diagnosis , Adult , Benzamides , Diagnosis, Differential , Humans , Imatinib Mesylate , Male , Myelodysplastic Syndromes/diagnosis , Piperazines/therapeutic use , Pyrimidines/therapeutic use , Remission InductionABSTRACT
The incidence of isolated extramedullary disease (EMD) following allogeneic hematopoietic stem cell transplant (allo-HSCT) for chronic myelogenous leukemia (CML) is not fully known. One review found the incidence of isolated myeloid EMD, or granulocytic sarcoma (GS), in an allo-HSCT treated CML/myelodysplastic subgroup to be just 0.22%. The incidence of lymphoid EMD in similar patients is extremely rare with only two cases reported in the literature. While the etiology of EMD in the post-transplant setting is not entirely clear, there may be inefficacy of immune surveillance function outside of the bone marrow cavity. Isolated CML GS following allo-HSCT carries a median interval to bone marrow relapse between 7 and 10 months and a median survival of 12 months. Less is known about lymphoid EMD. The treatment in these cases is ill defined with modalities ranging from involved field radiation to second allo-HSCT. We present a case of isolated pancreatic lymphoid EMD diagnosed 15 months after allo-HSCT for CML. Our patient was also treated with withdrawal of his immunosuppressive regimen. Unfortunately, at just over 4 months following pancreatic resection, he developed systemic relapse and died. While EMD can occur anywhere in the body, CML associated pancreatic EMD is not previously reported.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Pancreatic Diseases/etiology , Stem Cell Transplantation/adverse effects , Adrenal Cortex Hormones/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Cells/pathology , Fatal Outcome , Graft vs Host Disease/pathology , Humans , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Pancreatectomy , Pancreatic Diseases/pathology , Pancreatic Diseases/surgery , Recurrence , Transplantation, Homologous/adverse effectsABSTRACT
The oxygenation-linked, allosteric interactions of erythrocytic organic phosphates and urea with the haemoglobin (Hb), and the functional significance of the Hb multiplicity, were studies in an elasmobranch, Squalus acanthias. The autochthonous red cell nucleoside triphosphates (NTP) ATP and GTP (guanosine triphosphate) strongly depress O2 affinity of the stripped (cofactor-free) Hb and increase cooperativity in O2 binding. As previously found in teleost Hbs, GTP exerts a greater effect than ATP at the same concentration. Urea, in contrast, increases O2 affinity and depresses cooperativity. It also antagonizes the modulator effectivity of NTP at physiological NTP/Hb concentration ratios. Deoxygenation of the Hb raises blood pH. This Haldane effect contrasts with earlier findings for Pacific specimens, but accords with the presence of a Bohr effect (phi = delta log P50/delta pH). S. acanthias Hb resolves into six main components (three pairs) on the basis of isoelectric point. There is no evidence for radical functional differentiation as found in teleosts with electrophoretically anodal and cathodal Hb components. The physiological implications of the findings and the possible molecular mechanisms basic to the NTP and urea effects are discussed.
