Alžběta Zavřelová

Pneumocystis pneumonia during medicamentous treatment of Cushing's syndrome--a description of two cases.

Only a few cases of pneumocystis pneumonia (PCP) in Cushings syndrome have been published in the literature so far. In the majority of these patients, the pneumonia occurred after reduction of the hypercortisolism with medicamentous treatment. We report two cases of PCP during conservative treatment of hypercortisolism. We describe clinical, imaging and laboratory findings in two patients and review published cases of pneumocystits pneumonia in Cushings syndrome. A 60-year-old woman and 20-year-old man with Cushings syndrome due to ectopic ACTH syndrome were treated at our department. Both developed pneumocystis pneumonia early after treatment with ketoconazole and ethomidate bromide had been introduced and the levels of cortisol rapidly decreased. PCP prophylaxis in patients with high cortisolemia should be started before treatment of hypercortisolism in current practice. Gradual lowering of plasma cortisol should also reduce the risk of infection by Pneumocystis jiroveci.

Case 3-2011: Invasive mucormycosis (zygomycosis) after bone marrow transplantation in a 26-year-old man with relapsing acute myeloid leukaemia.

Patient J.S., 26-year-old man, was first seen by his general practitioner because of chest pain, dyspnoea, weakness, vomiting, headache, dizzines and recent fever on January 29th 2010. Blood samples were taken and revealed leukocytosis 149 × 109/L. His previous medical history was unremarkable. He was immediately referred to our University Hospital with suspicion of acute leukaemia. On admission in the evening of the same day, his leukocyte blood count was already 248 × 109/L and mild anaemia and thrombocytopenia was present. The diagnosis of acute myeloid leukaemia (myelomonocytic according to WHO classification) was made. There also was a severe syndrome of leukostasis on admission, and the patient developed acute myocardial infarction and respiratory insufficiency. Immediate leukoreduction with leukapheresis and hydroxyurea was started. When leukocyte count was reduced below 50 × 109/L chemotherapy with intermediate dose cytarabine (total dose 24 g) and idarubicin (total dose 70 mg) was started. Despite drastic leukoreduction our patient developed systemic inflammatory response syndrome (SIRS) with multiorgan failure reflecting tumor lysis syndrome following chemotherapy. He received artificial ventilation for respiratory failure caused by diffuse alveolar haemorrhage, and continuous renal replacement therapy for renal failure was started. The course of his disease was further complicated by shock, disseminated intravascular coagulation and liver failure. With full supportive care and corticosteroids patient was stabilised and eventually, after 5 days, he was successfully extubated and his renal and liver function recovered. Unfortunately, the induction chemotherapy did not lead to remission of the leukaemia. Patient received reinduction chemotherapy with FLAG IDA regimen (fludarabine, cytarabine, idarubicin, G-CSF) on March 6th. The remission was not achieved once again and another reinduction with HAM chemotherapy (cytarabine, mitoxantrone) was instituted on April 18th. Finally, there was a complete remission on bone marrow examination on May 27th. Patient obtained consolidation treatment (again HAM chemotherapy) and was scheduled for allogeneic stem cell transplantation. On August 18th he was admitted for allogeneic peripheral blood stem cell transplantation. His donor was unrelated woman with 3 mismatches (7/10). Unfortunately, before transplantation his bone marrow examination revealed a relapse of acute leukemia, and we decided to proceed to fully ablative approach enforced with cytarabine and mitoxantrone. The remission with 96% donor chimerism was achieved on September 29th. After transplantation he developed grade 3 skin acute graft versus host disease and BK virus hemorrhagic cystitis. Graft versus host disease resolved with corticosteroids treatment and hemorrhagic cystitis with reduced immunosupression. On November 11th, second relapse of the disease was diagnosed. The patient received chemotherapy and again developed multiorgan failure with respiratory failure and was artificially ventilated. He recovered and still cytopenic he refused further hospital stay and was discharged. Voriconazol was used as a prophylaxis during neutropenia. He was admitted shortly afterwards with the clinical signs of sepsis. Physical examination and X-ray revealed left side pneumonia and pericarditis. On pulmonary high resolution CT scan (HRCT), there was bilateral pneumonia with atypical pattern (Fig. 1). He also complained of vision disturbance, and an ischemic lesion in the occipital area on brain CT scan was found (Fig. 2). Lumbar puncture did not reveal any pathogen. Immediate treatment with antibiotics (meropenem and amikacin) and amphotericin B in combination was started. The patient died within 2 days (November

Treatment of Multifocal Multisystem BRAF Positive Langerhans Cell Histiocytosis with Cladribine, Surgery and Allogenic Stem Cell Transplantation

Langerhans cell histiocytosis (LCH) is a very rare disease in adults and as well a very rare cause of sellar expansion. The clinical presentation can be heterogeneous, from a single bone lesion to potentially fatal, widespread disease. We describe the difficulties with the diagnosis and treatment of LCH as well as successful treatment with cladribine chemotherapy and allogeneic stem cell transplantation.

Fifteen years of single center experience with stem cell transplantation for multiple myeloma: a retrospective analysis.

INTRODUCTION Autologous stem cell transplantation (ASCT) became standard of care for patients with multiple myeloma (MM) under the age of 65 years. We routinely perform ASCT for newly diagnosed MM since 1996 in our department. PATIENTS AND METHODS We retrospectively analyzed all 285 transplants in 185 patients done for MM from January 1996 till December 2010. We analyzed overall survival (OS) and progression-free survival (PFS) regarding conditioning, stage, complete or very good partial remission (CR, VGPR) achievement, renal impairment, single vs. double transplant. RESULTS Estimated 10-years survival of the whole set of patients is 39% (median survival 95 months). Patients with renal impairment show same OS as those without (p = 0.22). Patients show similar overall survival and event free survival regardless of type of transplant. We observed better outcome in terms of overall survival in patients treated with new drugs (p = 0.0014). Reaching CR or VGPR was not translated into better OS (p = 0.30) and EFS (p = 0.10). Also stage of the disease and whether single or double transplant was used did not make any significant difference in the outcome. CONCLUSION Stem cell transplantation greatly improved outcome of patients with MM. Poor outcome of allogeneic transplantation in our group of patients is related to high transplant related mortality (20% vs. 0%) and unexpected high relapse rate. There is a trend towards better survival, when new drugs are incorporated at any time in the course of the disease. This fact supports hypothesis that use of these drugs with ASCT should translate into better long-term outcome.

Case 2-2012: a 57-year-old woman with post-transplant lymphoproliferative disorder after allogeneic stem cell transplantation for primary myelofibrosis.

allogeneic haematopoietic stem cell transplantation (hsCt) has become a major life sustaining treatment for haematopoietic disorders, and it is the preferred treatment option for selected patients with idiopathic myelofibrosis. Still, it may be accompanied by various complications. here, we present a case of epstein-Barr virus (eBv) – associated post-transplant lymphoproliferative disorder (Ptld), i.e. an infection-induced malignant proliferation following hsCt.