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BCR-ABL1 negative atypical chronic myeloid leukemia (aCML) is a rare type of myeloproliferative / myelodysplastic syndrome characterized by leukocytosis and proliferation of dysplastic neutrophilic precursors in the absence of positivity for the BCR-ABL1 fusion gene. We report a 66-year-old woman and a 57-year-old man with aCML, who initially presented with general malaise and weight loss, associated with anemia, thrombocytopenia, and leukocytosis with left shift and dysplasia in the neutrophil series. Both evolved unfavorably after admission and died a few days later due to multiple organ failure.

Background The treatment of choice of newly diagnosed multiple myeloma (NDMM) is an induction with proteasome inhibitors followed autologous stem cell transplantation (HSCT). Since 2013, the treatment of these patients in the public system is based on CTD (cyclophosphamide, thalidomide, and dexamethasone). Aim To evaluate the response rates achieved with CTD, and the results of HSCT in patients with NDMM in the public setting. Material and Methods Data from patients considered as candidates for HSCT from different centers of the National Adult Antineoplastic Drug Program (PANDA, for its acronym in Spanish), diagnosed between 2013 and 2017, was analyzed. The response to treatment of first and second lines of treatment was evaluated, in addition to the results of HSCT. An optimal Response was defined as the sum of strict complete remission, complete remission and very good partial response (sCR, CR and VGPR). Results One hundred and seventy-seven patients were analyzed, 54% women, and 53% with IgG multiple myeloma. Information about the international staging system was retrieved in 127 patients (71%). Seventeen percent were ISS I, 22% in ISS II and 32% ISS III. CTD was used as first treatment in 106 patients (60%), and cyclophosphamide, bortezomib and dexamethasone (CyBorD) in 13 (7%). As first line, CTD had an overall response of 50.9%, and CyBorD of 76.9%. Thirty patients were treated with bortezomib as second line treatment. Forty patients (22%) underwent HSCT. The 5-year Overall Survival (OS) in transplanted patients and non-transplanted patients was 100 and 62% respectively (p < 0.01). Conclusions The response rate achieved by CTD in these patients is suboptimal. The response to CyBorD was better.

Background The treatment of choice of newly diagnosed multiple myeloma (NDMM) is an induction with proteasome inhibitors followed autologous stem cell transplantation (HSCT). Since 2013, the treatment of these patients in the public system is based on CTD (cyclophosphamide, thalidomide, and dexamethasone). Aim To evaluate the response rates achieved with CTD, and the results of HSCT in patients with NDMM in the public setting. Material and Methods Data from patients considered as candidates for HSCT from different centers of the National Adult Antineoplastic Drug Program (PANDA, for its acronym in Spanish), diagnosed between 2013 and 2017, was analyzed. The response to treatment of first and second lines of treatment was evaluated, in addition to the results of HSCT. An optimal Response was defined as the sum of strict complete remission, complete remission and very good partial response (sCR, CR and VGPR). Results One hundred and seventy-seven patients were analyzed, 54% women, and 53% with IgG multiple myeloma. Information about the international staging system was retrieved in 127 patients (71%). Seventeen percent were ISS I, 22% in ISS II and 32% ISS III. CTD was used as first treatment in 106 patients (60%), and cyclophosphamide, bortezomib and dexamethasone (CyBorD) in 13 (7%). As first line, CTD had an overall response of 50.9%, and CyBorD of 76.9%. Thirty patients were treated with bortezomib as second line treatment. Forty patients (22%) underwent HSCT. The 5-year Overall Survival (OS) in transplanted patients and non-transplanted patients was 100 and 62% respectively (p0.01). Conclusions The response rate achieved by CTD in these patients is suboptimal. The response to CyBorD was better.

We report a 58 year-old-man without comorbid conditions, with a history of two months of weight loss, malaise and headache. His initial laboratory analysis showed leukocytosis of 16,100/mL with 65% eosinophils and an absolute eosinophil count of 10,465/mL. Both bone marrow biopsy and aspirate showed infiltration by mature appearing eosinophils. Treatment was started with hydroxyurea, associated with prednisone without satisfactory decrease in the eosinophil count. Polymerase chain reaction showed the presence of the gene fusion product FIP1L1/PDGFRA. Imatinib therapy was initiated, resulting in a rapid and progressive reduction in the absolute eosinophil count, with normalization at the second week of treatment. The incidence of the myeloproliferative variant causing hypereosinophilic syndrome is rare. However, the dramatic response to imatinib emphasizes the need to study the presence of the fusion product FIP1L1/PDGFRA in all patients with eosinophilia of unknown etiology.

The initial presentation of Hodgkin lymphoma with liver involvement is rare. In these patients, the standard first-line therapy with ABVD (Adriamycin, Bleomycine, Vinblastine, Dacarbazine) imply an additional risk for liver toxicity. We report a 64-year-old woman who presented with jaundice, choluria, malaise and weight loss. In the initial evaluation she had jaundice and palpable groin lymph nodes. An obstructive biliary disease was ruled out with magnetic resonance imaging studies. A lymph node biopsy showed a Hodgkins lymphoma, Mixed-cellularity subtype. Considering the liver dysfunction, an alternative scheme of chemotherapy with dexamethasone, gemcitabine and cisplatin (GDP) was administered. After 4 cycles, a significant improvement in liver hepatic function tests was reached and a conventional chemotherapy (ABVD) was begun. While the literature provides some low toxicity protocols for patients with liver involvement, favorable results of our clinical case report allows us to postulate GDP as an alternative for salvage therapy in these patients.

We report a 67-year-old man presenting with abdominal pain of acute onset, pallor, jaundice and behavioral changes after ingestion of fava beans. In the initial evaluation he appeared acutely ill and had resting dyspnea, edema and jaundice. His initial laboratory assessment disclosed azotemia, elevated lactate dehydrogenase levels, a low hemoglobin concentration (4.9 /dL) and a high corrected reticulocyte count (4,7%) with negative direct and indirect Coombs' test. The patient was transferred to the ICU, where he received support therapy with hemodialysis, mechanical ventilation, vasoactive drugs and transfusions of packed red cells. The evolution after 1 month was favorable and he was discharged without anemia and with normal renal function. Three months after discharge, the glucose-6-phosphate-dehydrogenase screening study did not demonstrate detectable enzymatic activity.

POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. Glomeruloid hemangioma is a typical skin change pathogenetically related with elevated levels of Vascular Endothelial Growth Factor (VEGF). We report a 69 year-old woman that presented cachexia associated with diabetes, hypothyroidism and severe sensitive motor polyneuropathy. Her skin changes included hyper pigmentation, acrocyanosis and glomeruloid hemangioma. The subsequent study revealed a monoclonal gammopathy lambda type; a unique lytic vertebral lesion and a clonal plasma cell proliferation. Treatment with prednisone 0.5 mg/kg and melphalan 0,25 mg/kg in cycles of 4 days every 4 weeks was started, but the patient was lost from follow up.

POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. Glomeruloid hemangioma is a typical skin change pathogenetically related with elevated levels of Vascular Endothelial Growth Factor (VEGF). We report a 69 year-old woman that presented cachexia associated with diabetes, hypothyroidism and severe sensitive motor polyneuropathy. Her skin changes included hyper pigmentation, acrocyanosis and glomeruloid hemangioma. The subsequent study revealed a monoclonal gammopathy lambda type; a unique lytic vertebral lesion and a clonal plasma cell proliferation. Treatment with prednisone 0.5 mg/kg and melphalan 0,25 mg/kg in cycles of 4 days every 4 weeks was started, but the patient was lost from follow up.