Adrenal glands are one of the organs where malignancies frequently metastasize. However, primary malignancies of the adrenal glands are rare. Primary adrenal lymphomas (PAL) account for less than 1% ...of extranodal lymphomas. It is seen bilaterally in 75% of cases. The most common subtype is diffuse large B-cell lymphoma.
A 57-year-old male patient with no known history of disease applied to an external center with complaints of weight loss (13 kg, more than 10% of body weight), tremors, loss of appetite and lower back pain. As a result of the evaluations made at an external center, thorax CT showed bilateral adrenal masses. PET CT was taken with the preliminary diagnosis of malignancy; In the right adrenal gland, approximately 11.6 × 8.1cm (SUVmax: 25.6) and in the left adrenal gland, approximately 10.1 × 7.2cm (SUVmax: 24.4) in size, heterogeneous dense hypermetabolic solid mass sections with necrotic areas were seen and left paraaortic (SUVmax: 11.7) lymph node with dimensions of 1.6 × 1.2 cm and a few mildly-intensely hypermetabolic lymph nodes were observed in the interaortocaval chain. Primary malignancy storage was evaluated in the foreground of dense hypermetabolic mass regions of heterogeneous structure defined in both adrenal glands. After pheochromocytoma was diagnosed, the patient was referred to our clinic after the tru-cut pathology performed on the mass lesions in the right adrenal gland revealed that the morphological and histochemical findings were consistent with diffuse large B-cell lymphoma.
The patient's vital signs were stable upon admission. In the hemogram, Hgb: 9.7g/dL Hct: 31.2% MCV: 96 fL Platlet: 126.000 / mm3. In biochemistry, creatinine:1.83mg/dL urea:80 mg/dL Na:139 mmol/L K:4.36 mmol/L Ca:10.2mg/dL uric acid:9.4mg/dL LD:343U/L. There was no sign of adrenal insufficiency other than dehydration. The patient was started on hydration and allopurinol treatments. During follow-up, urea and creatinine levels decreased to normal limits. The patient's basal cortisol was 12.02 µg/dL and ACTH was 83.8ng/L. Low-dose (1µg) ACTH test was performed on the patient, for whom chemotherapy was planned for his primary disease, with the preliminary diagnosis of adrenal insufficiency. The patient's cortisol levels were detected as 8.23-10.35-8.93-9.75 µg/dL at 30-60-90 and 120 minutes, respectively, and hydrocortisone treatment was started with the diagnosis of adrenal insufficiency. During the follow-up of the patient, R-CHOP chemotherapy was started, and since the patient had prednisolone in the chemotherapy course, hydrocortisone was discontinued during chemotherapy and isolated prednisolone treatment was given. Central Nervous System involvement was detected in the cerebrospinal fluid during intrathecal (IT) chemotherapy (Mtx, Dexamethasone, Cytosine Arabinosine). Intrathecal therapy was initially administered 3 times a week and subsequently twice a week. Since no cells were detected in the cytocentrifuge, intrathecal chemotherapy was given 4 times. After 6 cycles of R-CHOP chemotherapy, the patient underwent Autologous peripheral stem cell transplantation because of high risk disease in December 2023. The patient, whose general condition is good during follow-up, is currently being followed in remission under replacement dose hydrocortisone treatment.
Burkitt lymphoma is an aggressive type of B-cell lymphoma that is usually seen in the pediatric and young adult population and characteised with myc gene translocation. This disease manifests itself ...with rapidly growing abdominal mass and systemic sign and symptoms. However, atypical involvements, such as isolated cranial involvements, face both diagnostic and therapeutic challenges, especially in older age groups. Although isolated cranial involvement of Burkitt's lymphoma is rare in older patients, this case report emphasizes the challenges in clinical practice.
A 67-year-old female patient was taken with complaints of headache and vomiting in June 2022. An MRI scan revealed a mass measuring 3.3 × 2.8 × 1.5 cm in the left temporal region. Upon this finding, the patient was referred to the neurosurgery department and the mass was surgically removed. As a result of the pathological examination resulting from the operation, CD10, CD20 were diffusely positive; BCL2 negative; BCL6 positive; C-MYC 70% positive; Kİ67 is 100% positive and confirms Burkitt's Lymphoma. In the PET-CT scan performed for the staging of the patient, reticular dense growths and irregular growth FDG uptakes in ground glass density areas were observed in the medial posterobasal segment of the lower lobe of two lungs and in the anterior segment of the upper lobe of the left lung. In the mediastinal area, increased degrees of FDG uptake were detected in bilateral lower paratracheal and subcarinal lymph nodes. These findings were evaluated as a potential infectious event. While there were no findings in hemogram and biochemical pathological tests, HbsAg positivity was detected but no active disease was found.Prophylactic intrathecal(IT) treatmentwas also recommended for the disease, which started to systemic chemotherapy, but IT chemotherapy was rejected.. In subsequent MRI examinations, the defect formed after craniotomy in the left temporofrontoparietal region and fluid collection in the calvarium were observed, while no residue or recurrence was observed in the operation area.However, a lesion measuring 2 × 3 cm in size was detected in the left parietal at the vertex level, which was primarily considered a fibroma and showed marked hypointenses and heterogeneous contrast enhancement in all sections.
In the evaluation PET-CT performed after four cycles of the R-HYPERCVAD regimen, a mild increase in metabolic activity was observed in the mediastinal lymph nodes, but this was consistent with inflammatory processes, and no signs of recurrence or metastasis were found in other parts of the body. Despite these findings, which were accepted as a response to treatment, the planned OKIT treatment was not accepted by the patient and their relatives.
After completing the seventh course of treatment, the patient presented to the emergency room with altered consciousness and recurrent headaches. Antieodema treatment was applied to the patient who was diagnosed with brain edema, but the recommended advanced chemotherapy and full cranial radiotherapy were rejected. In December 2023, the patient was re-admitted with symptoms of brain edema and shingles zoster infection was observed, and the patient died after his condition worsened despite symptomatic treatment.
This case report highlights the rarity of advanced age Burkitt lymphoma with isolated cranial involvement and the diagnostic and therapeutic difficulties of this condition. Our patient exhibited atypical involvement of an aggressive B-cell lymphoma that usually occurs in childhood and young adults and is characterized by myc gene translocation. The disease, which usually manifests itself with an abdominal mass and systemic symptoms, is rare to show isolated cranial involvement, and this requires us to re-evaluate the diagnosis and treatment strategies in our clinical practice. In this case, although the patient's symptoms and radiological findings initially suggested a typical brain tumor, pathological examination confirmed the diagnosis of Burkitt's lymphoma. During the patient's treatment process, the importance of systemic chemotherapy and prophylactic intrathecal treatment became evident. However, rejection of various treatment options by patients and their relatives may negatively affect the effectiveness of the treatment and patient survival. This case highlights the rarity of Burkitt lymphoma with isolated cranial involvement in older age patients and the challenges and important lessons encountered in the diagnosis and treatment of these atypical presentations.
Multiple myeloma is a hematological malignancy that develops as a result of clonal proliferation of plasma cells and progresses with remissions and relapses. It is clinically characterized by many ...symptoms and signs such as osteolytic bone lesions, hypercalcemia, renal dysfunction, hypergammaglobulinemia and anemia. However, involvement of the central nervous system, especially the leptomeningeal/cranial region, is a rare and prognostically important form of relapse of the disease. Nervous syste
Purpose
To investigate serum levels of interleukin (IL)-12 (Th1 cytokine), IL-27 (an immunomodulatory cytokine), IL-4 (suppressor of Th1-cell growth), IL-13 (a stimulatory signal for Th2 cytokines), ...and IL-33 (an epithelial cell-derived cytokine) and their relations with the disease activity in Behcet’s Disease (BD).
Methods
Four groups, each composed of 20 participants were enrolled in the study; active ocular BD (Group-A), ocular BD in remission (Group-B), nonocular BD in remission (Group-C) and healthy controls (Group-D). IL levels were compared between the study groups and their correlation with the disease activity parameters were analyzed.
Results
IL-13 and IL-33 were higher in Group-A. IL-27 was lower in all BD groups. Additionally, IL-13 and IL-33 levels were positively correlated with disease activity parameters.
Conclusion
These findings show Th2 dominance in the active phase of BD. Besides, decreased levels of IL-27, and presumably, its protective anti-inflammatory effect in all study groups may exert a new pathologic finding in BD.
The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH ...patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17–77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (
n
= 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7–183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.
Brucellosis is a worldwide zoonotic disease associated with hemolytic complications, including thrombotic microangiopathy (TMA) and hemolytic anemia. Autoimmune hemolytic anemia (AIHA) is a rare ...clinical presentation of this disease. In this report, we describe the cases of two patients with brucellosis who presented with Coombs-positive AIHA. We also include a review of the literature on the hemolytic complications of brucellosis. Both patients were successfully treated with a combination of doxycycline and rifampicin in addition to steroids. In the medical literature, there are several cases of TMA associated with brucellosis, although only a few cases of Coombs test-positive AIHA have been reported. Antibiotic therapy is the mainstay of treatment, and the selection of antibiotics and duration of treatment do not differ between brucellosis patients with and without hemolysis. Although rare, the potential for brucellosis should always be kept in mind in patients who present with hemolysis, especially those living in areas where brucellosis is endemic.
Low cholesterol levels may be accompanied by solid tumors or hematological malignancies such as multiple myeloma. Decreased cholesterol levels have been reported in some experimental studies about ...chronic lymphocytic leukemia (CLL). It may be associated with tumoral cell metabolism. Herein, we examine blood lipid profiles of patients with newly diagnosed CLL (284 male, 276 female, mean age 64 ± 11 years) as defined by National Cancer Institute criteria. The control group consisted of 71 healthy subjects with mean age 55 ± 9 years (28 male, 43 females). 60% of patients with Binet A, while 25% were Binet C. Decreased levels of total cholesterol, high density lipoprotein (HDL) and low density lipoprotein (LDL) were observed in patients with CLL than control group (p < 0,001). There was no statistical significance between CLL and control group for triglycerides (TG) and very low density lipoprotein (VLDL), also between HDL-C, VLDL, TG and grades. Cholesterol may metabolized by abnormal lymphocytes in CLL patients.
Objective:To evaluate the febrile neutropenic patients with hematological malignancies hospitalized in hematology clinic with poor hygiene standards.Methods:A total of 124 patients with hematological ...malignancies(69 male,55 female)hospitalized in hematology clinic with poor hygiene conditions depending on hospital conditions,between January 2007 and December 2010,were evaluated,retrospectively.Results:In this study,250 febrile neutropenia episodes developing in 124 hospitalized patients were evaluated.Of the patients,69 were men(56%)and 55 women(44%).A total of 40 patients(32%)had acute myeloid leukemia,25(20%)acute lymphoblastic leukemia,19(15%)non-Hodgkin’s lymphoma,10(8%)multiple myeloma,and 8(8%)chronic myeloid leukemia.In our study,56 patients(22%)were diagnosed as pneumonia,38(15%)invasive aspergillosis,38(15%)sepsis,16(6%)typhlitis,9(4%)mucormycosis,and 4(2%)urinary tract infection.Gram-positive cocci were isolated from 52%(n=20),while Gram-negative bacilli 42%(n=16)and yeasts from 6%(n=2)of the sepsis patients,respectively.The most frequently isolated Gram-positive bacteria were methicillin-resistant coagulase-negative staphylococci(n=18),while the most frequently isolated Gram-negative bacteria was Escherichia coli(n=10).Conclusions:Febrile neutropenia is still a problem in patients with hematological malignancies.The documentation of the flora and detection of causative agents of infections in each unit would help to decide appropriate empirical therapy.Infection control procedures should be applied for preventing infections and transmissions.
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