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Diffuse large B cell lymphoma (DLBCL) constitutes the most frequent subtype of all non-Hodgkin's lymphomas. DLBCL is an aggressive disease and extranodal involvement is seen in approximately 30% of patients and most common extranodal sites are gastointestinal tract and skin. Skin involvement may be either primary or secondary. Secondary cutaneous lymphoma has a worse prognosis. The case is here reported of a 56-year old male DLBCL patient with cutaneous lesions and aggressive clinical course. The patient had no skin lesions at diagnosis and during follow up and treatment period, skin, cerebrospinal fluid and bone marrow involvement was occurred. Salvage chemotherapy and autologous stem cell transplantation was planned but the patient died before the second cycle of salvage chemotherapy. In contrast to primary cutaneous lymphoma, which tends to be more indolent, secondary skin involvement is associated with unfavourable prognosis. In conclusion it should be kept in mind that skin can be involved in lymphoma patients and in these cases, skin biopsy should be performed rapidly. • Diffuse large B cell lymphoma is an aggressive disease and extranodal involvement is seen in approximately 30% of patients. • Most common extranodal sites are gastointestinal tract and skin. Skin involvement may be either primary or secondary. • Secondary cutaneous lymphoma has a worse prognosis. • Salvage chemotherapy followed by autologous stem cell transplantation is the best treatment choice. • Extramedullary involvement is associated with poor prognosis not only in lymphomas but in all hematological malignancies. [ABSTRACT FROM AUTHOR]

Hairy cell leukemia (HCL) is a rare mature B-cell malignancy that is primarily treated with purine analogues. However, relapse remains a significant challenge, prompting the search for alternative therapies. The BRAF V600E mutation prevalent in HCL patients provides a target for treatment with vemurafenib. This multicenter retrospective study included nine patients with relapsed/refractory (R/R) HCL from six different centers. Patient data included demographics, prior treatments, clinical outcomes, and adverse events. Patients received different treatment regimens between centers, including vemurafenib alone or in combination with rituximab. Despite the differences in protocols, all patients achieved at least a partial response, with seven patients achieving a complete response. Adverse events were generally mild with manageable side effects. The absence of myelotoxic effects and manageable side effects make BRAF inhibitors attractive, especially for patients ineligible for purine analogues or those with severe neutropenia. Single agent vemurafenib or in combination with rituximab appears to be a promising therapeutic option for R/R HCL. Further research is needed to establish standardized treatment protocols and to investigate long-term outcomes. • Evaluate the use of vemurafenib for relapsed/refractory HCL with BRAF V600E mutation. • There are potential benefits of vemurafenib treatment, particularly in patients who are ineligible for purine analogues or who have severe neutropenia. • Further research is needed to establish standardized treatment protocols for vemurafenib monotherapy or vemurafenib in combination with rituximab in HCL. [ABSTRACT FROM AUTHOR]

Transverse myelitis is a quite rare complication of hematopoietic stem cell transplantation. The case is here reported of a 49 year old male with diffuse large B cell lymphoma in complete remission who developed transverse myelitis after autologous stem cell transplantation. The patient presented with numbness and sensory loss of the bilateral lower extremities and difficulty in urinating on the 20th day after cell transplantation. Millimetric hyperintensity was detected in the C5-C6 and T2-T5 segments of the spinal cord on cervical and thoracic vertebral magnetic resonance imaging. Treatment was initiated of pulse steroid and intravenous immunoglubulin followed by plasmapheresis and cyclophosphamide due to inadequate response. The patient then started a rehabilitation program and was discharged in the 9th month after stem cell transplantation when most of the symptoms were relieved. To the best of our knowledge, this is the first case reported in literature of TM development after autologous stem cell transplantation. [ABSTRACT FROM AUTHOR]

The case is here presented of a 70-year old male patient with rare coexistence of Kaposi Sarcoma and resistant Thrombotic Thrombocytopenic Purpura (TTP). The Kaposi lesions were determined before the diagnosis of TTP and were exacerbated after receiving TTP-associated immunosuppressive therapy, in particular associated with rituximab. TTP in this case was resistant to conventional therapies such as steroid and plasma exchange and current immunosuppressive (rituximab, cyclophosphamide, vincristin) treatments. Novel treatment agents consisting of bortezomib and eculizumab given to the patient were also ineffective. To the best of our knowledge, this case presents the first case of coexistence of TTP and Kaposi sarcoma from Turkey and the challenge of refractory TTP management. [ABSTRACT FROM AUTHOR]

Hemophilia is a hereditary disease with impaired blood coagulation due to a genetic deficiency of blood coagulation factors. The development of inhibitors further complicates the course of the disease and management. The case is here reported of a haemophilia patient who presented with coexisting development of high titer inhibitor with Gastrointestinal Stromal Tumor (GIST) diagnosis and was admitted with upper gastrointestinal system bleeding. The patient had no prior history of inhibitor presence. During all procedures including surgery, excellent hemostasis was achieved with rFVIIa treatment and no hemorrhagic complication was observed. To the best of our knowledge, this constitutes the first reported case of GIST associated with inhibitor development in a hemophilia A patient. [ABSTRACT FROM AUTHOR]

Hemophagocytic Lymphohistiocytosis (HLH) is a reactive disorder of the mononuclear phagocytic system characterized by increased histiocytic proliferation, activation and hemaphagocytosis. The underlying etiology may be genetic (primary) or acquired (secondary). Secondary causes include drugs, autoimmune diseases, malignancies and infections of which EBV is the most common. A 28-year old male patient who was a shepherd with no known concomitant comorbid disease was admitted to the Emergency Department with the complaints of abdominal pain, fever, severe fatigue. Physical examination revealed high fever, hepatosplenomegaly and laboratory examination revealed pancytopenia, hyperferritinemia and hypertriglyceridemia. Hemophagocytes were observed in the bone marrow biopsy and the patient was diagnosed as HLH. The patient was treated with cyclosporine A, dexamethasone, intravenous immunoglobulin (IvIg) and etoposide according to the HLH 2004 protocol. Coxiella burnetii was detected in the serological evaluation of the etiology and doxycycline was added to the current treatment. Fever was controlled in the second week of the treatment and the patient was discharged after complete recovery of the cytopenia in the fourth week. In the outpatient setting, treatment was completed in 8 weeks and follow-up of the patient is still ongoing without medication. To the best of our knowledge, this is the first case from Turkey of HLH secondary to Q-fever which was treated and managed successfully. Since the mortality of HLH is quite high, the etiology should be determined as soon as possible to be able to provide appropriate treatment. [ABSTRACT FROM AUTHOR]