A rare case of pulmonary lymphomatoid granulomatosis complicated with venous thrombosis.

Lymphomatoid granulomatosis (LG) is Epstein-Barr virus associated and aggressive B cell lymphoproliferative disease. The most common sites of involvement are lungs, skin, kidneys, liver and central nervous system. The clinical presentation of pulmonary LG may mimic infectious diseases, malignancies or vasculitis. While treatment approach of low grade disease is watch and wait, patients with advanced stage require aggressive treatment with chemotherapy. Patients with hematological malignancy as well as solid tumors are at increased risk of venous thromboembolic events (VTE). We reported here in a case of pulmonary LG who was complicated with VTE during treatment with chemo-immunotherapy After 4 cycles of R-CHOP, she achieved complete remission for LG and was followed up without relapse for 2 years. She was anticoagulated with Low-Molecular-Weight Heparin (LMWH) during chemotherapy period, and the thrombus improved over the next several weeks. While on this paper written, patient completed her pregnancy successfully under anticoagulation prophylaxis.

A rare case of diffuse large B-cell lymphoma-associated hemophagocytic lymphohistiocytosis.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. Herein, we aimed to report a diffuse large B-cell lymphoma (DLBCL) case that was presented as HLH. CASE REPORT: A 32-year-old man presented to a hospital with complaints of vomiting, nausea and diarrhea in October 2019. Fever and hepatosplenomegaly was detected in physical investigation. Bone marrow aspiration investigation revealed the hemaphagocytosis. HLH-2004 protocol was started for hemophagocytosis. Whole body magnetic resonance imaging (MR) revealed no lymphadenopathy. Bone marrow biopsy revealed high-grade B-cell lymphoma, favoring DLBCL. There were no pathologic cells in lumber puncture investigation. MANAGEMENT AND OUTCOME: He was diagnosed with secondary hemaphagocytic syndrome due to DLBCL, and chemotherapy was switched to rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (R-EPOCH) regimen. After three cycles of R-EPOCH chemotherapy regimen, complete remission was confirmed with positron emission tomography-computerised tomography (PET-CT) scan. DISCUSSION: Our patients' findings are suitable for six out of eight criteria of hemaphagocytic syndrome. The H-score of our patient was more than 250, reflecting the >99% probability of HLH syndrome. Compatible with literature knowledge, our patient had responded very well to etoposide-containing regimens. In our patient, no lymphadenopathy was detected by physical examination or MR scan, and the diagnosis of DLBCL was only made by the result of bone marrow investigation. In conclusion, herein, we have reported a DLBCL case that had presented with HLH, and clinicians should be aware that B-cell lymphomas may be the underlying cause of HLH.

Prognostic Factors in Patients with Low-Grade Nonhodgkin Lymphoma.

Low-grade Nonhodgkin lymphoma (LG-NHL) is characterized by indolent clinical course, which consist of marginal zone lymphoma (MZL), follicular lymphoma (FL), chronic lymphocytic leukemia/small lymphocytic lymphoma, lymphoplasmacytic lymphoma, waldenstrom macroglobulinemia (WM) as the most common subtypes. Factors affecting prognosis and treatment need in these patients have long been the subject of research. A retrospective study was conducted with patients diagnosed with LG-NHL in Hematology Departments of two centres between 2010 and 2018. At the time of diagnosis, demographic and disease characteristics, hematological and biochemical parameters were examined. Using these data, treatment requirements, response and survival rates were calculated. The effect of parameters on survival and need to treatment were analyzed. 93 LG-NHL patients were included in this study. 40 (43%) of these patients were MZL, 28 (30.1%) were FL and 25 (26.8%) were others. In comparison of patients required treatment with patients without treatment, there was significant difference among the number of comorbidity, platelet count, neutrophil count, disease subgroups and ferritin levels. Logistic regression analysis revealed that disease subgroup (other than MZL and FL) and ferritin levels were independent risk factors for need to treatment. Only ferritin level was found to be associated with overall survival. The current study demonstrated an association between serum ferritin levels and prognosis in patients with LG-NHL. Given that it is easily available and low-cost, the initial ferritin level can be used as a prognostic marker for patients with indolent lymphoma.

Ibrutinib-induced polyneuropathy: A case report.

INTRODUCTION: Ibrutinib is an oral irreversible inhibitor of Bruton's tyrosine kinase signaling. It is a well-tolerated agent with some side-effects, the most common of which are atrial fibrillation, diarrhea, upper respiratory tract infection, fatigue, nausea, rash and cytopenias. Most of these toxicities are mild, although some have a severe clinical course. CASE REPORT: The case is here reported of a chronic lymphocytic leukemia patient with ibrutinib-induced polyneuropathy. A 63-year-old male patient with chronic lymphocytic leukemia was given ibrutinib as a third line treatment regimen. After the 10th month of therapy he had progressive complaints of numbness and tingling in his legs. The patient was diagnosed as grade 3 sensorineural polyneuropathy with electromyography.Management and outcome: Considering that ibrutinib treatment may cause neuropathy, the ibrutinib was discontinued, after which the neuropathic complaints improved. However, the neck and axillary lymph nodes were enlarged and treatment had to be re-started therefore ibrutinib was started at a low dose and gradually increased. The patient is currently in the 14th month of treatment and still using ibrutinib without any severe side-effects. DISCUSSION: To the best of our knowledge, polyneuropathy as a unique side-effect of ibrutinib has not been previously reported. In addition to the well-known side effects of ibrutinib treatment, it should be kept in mind that polyneuropathy may also develop.

The incidence and risk factors of thrombosis and the need for thromboprophylaxis in lymphoma and leukemia patients: A 9-year single-center experience.

BACKGROUND: Patients with cancer are at increased risk of thromboembolic complications. There is no evidence-based guideline on the use of routine prophylaxis in hematological malignancies except in patients with multiple myeloma. The purpose of this study was to determine the incidence and risk factors of thrombosis and suggest a rationale for primary thromboprophylaxis in acute leukemia and lymphoma patients. PATIENTS AND METHODS: A retrospective study was conducted on newly-diagnosed acute leukemia and lymphoma patients who presented at our institution from November 2009 to March 2018. The study included a total of 157 patients with acute leukemia and 238 patients with lymphoma. The groups were analyzed to determine the incidence and risk factors of thromboembolic complications. RESULTS: The incidence of all thrombotic complications was 10.12% (40/395) including 11.4% (18/157) in patients with acute leukemia and 9.2% (22/238) in patients with lymphoma. The majority of events occurred in the first 6 months. Acute leukemia patients with thrombosis had a higher number of comorbidities than those without thrombosis (p < 0.05). Lymphoma patients with thrombotic complications had significantly higher beta-2-microglobulin and lactate dehydrogenase levels compared to those without thrombosis (p < 0.05). Major bleeding events developed in five (3.1%) acute leukemia patients and two (0.8%) lymphoma patients. All the major bleeding events occurred when the patients were thrombocytopenic (platelet < 50,000/mm3). CONCLUSIONS: Acute leukemia patients with any comorbidity and lymphoma patients with higher lactate dehydrogenase and beta-2-microglobulin are at high risk of developing thromboembolic complications. The prophylactic use of anticoagulant should be considered for those patients especially in the first 6 months.

An acute transverse myelitis attack after autologous stem cell transplantation: A rare case.

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.

Refractory Thrombotic Thrombocytopenic Purpura in a patient with Kaposi sarcoma.

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.