Synchronous Detection of Hairy Cell Leukemia and HIV-Negative Kaposi's Sarcoma of the Lymph Node: A Diagnostic Challenge and a Rare Coincidence

Hairy cell leukemia (HCL) is an uncommon chronic lymphoproliferative disorder and accounts for around 2% of all forms of leukemias. The association of HCL with other neoplasms, mainly non-Hodgkin's lymphomas, is well known. However, the simultaneous diagnosis of HCL and Kaposi's sarcoma is rare, with only few cases of such an association having been reported. We describe a 42-year-old male patient with a well characterized HCL and in whom HIV-negative Kaposi's sarcoma of the lymph node was detected

Minimal residual disease (MRD) detection with translocations and T-cell receptor and immunoglobulin gene rearrangements in adult acute lymphoblastic leukemia patients: a pilot study

Objective: Monitoring minimal residual disease has become increasingly important in clinical practice of ALL management. Break-point fusion regions of leukaemia related chromosomal aberrations and rearranged immunoglobulin (Ig) and T cell-receptor (TCR) genes are used as leukaemia specific markers in genetic studies of MRD.Material and Methods: A total of 31 consecutive patients with newly diagnosed ALL were screened for eligibility criteria. Of those 26 were included in the study. One patient with partial response following induction therapy and four patients who were lost to follow-up after induction were excluded from the study; thus 21 patients were evaluated for MRD by using polymerase chain reaction (PCR), heteroduplex analysis, sequencing and quantitative real time PCR techniques. Results: Chromosomal aberrations were detected in 5 (24%) of the patients and were used for MRD monitoring. Three patients had t(9;22) translocation, the other 2 had t(4;11) and t(1;19). MRD-based risk stratification of the16 patients analysed for Ig/TCR rearrangements revealed 3 low-risk, 11 intermediate-risk and 2 high-risk patients.Conclusion: MRD monitoring is progressively getting to be a more important predictive factor in adult ALL patients. As reported by others confirmed by our limited data there is a good correlation between MRD status and clinical outcome in patients receiving chemotherapy. The pilot-study presented here is the first that systematically and consecutively performs a molecular MRD monitoring of ALL patients in Turkey

Intradiploic Hematoma in a Hemophilic Patient: Hemophilic Pseudotumor of Calvarium

WOS: 000398059500025PubMed ID: 27610826

Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature

BACKGROUND: Extramedullary hematopoiesis (EH) is a compensatory phenomenon that results in the production of blood cell precursors outside the marrow in patients with chronic hemolytic anemia and ineffective erythropoiesis. EH usually involves the liver, spleen, and lymph nodes. It can also be found at paravertebral, intrathoracic, or pelvic locations. Intracranial EH is a rare entity and often asymptomatic but can sometimes lead to symptomatic tumor-like masses. Treatment options are controversial and include hypertransfusion, surgical excision, radiotherapy, and hydroxyurea (HU). STUDY DESIGN AND METHODS: Successful treatment of an intracranial EH mass with HU and blood transfusions in a beta-thalassemia major patient was discussed along with a review of the published literature on intracranial EH in thalassemia. RESULTS: In our patient, the extramedullary hematopoietic mass in the interhemispheric fissure showed a marked improvement after 6 months of HU and hypertransfusion therapy. In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes. CONCLUSION: There is no standard treatment approach in patients with symptomatic EH. HU with hypertransfusion regimen is a reasonable first-choice modality in treating intracranial EH masses

Considering Huntington's Disease and Neuroacanthocytosis in the Differential Diagnosis of Senile Chorea

Sporadic chorea that presents after the age of 50 years is called "senile chorea". Senile chorea is a rare entity with a wide differential diagnosis list. Causes of senile chorea include vascular and metabolic diseases, adverse events related to medications, hematologic and immune system diseases, genetic and sporadic neurodegenerative syndromes, and paraneoplastic disorders. Although the most common etiologies are vascular and metabolic disorders, neuroacanthocytosis, Wilson's and Huntington's disease are included in the differential diagnosis

The Impact of Prothrombotic Mutations on Factor Consumption in Adult Patients with Severe Hemophilia

The Impact of Prothrombotic Mutations on Factor Consumption in Adult Patients With Sever