Right Testicular Infarction Secondary to Spontaneous Testicular Vein Thrombosis in a Child: Case Report and Literature Review.

OBJECTIVES: To describe a case of right testicular vein thrombosis in a child and to review the literature describing similar cases and their management. METHODS: An extensive literature review of the English language literature was conducted using the following databases: PubMed, ScienceDirect and Google Scholar. RESULTS: Six cases were reported previously and summarized in this article. CONCLUSION: A 14-year-old boy who suffered from spontaneous right testicular vein thrombosis was treated with right orchiectomy and oral anticoagulation. We also reviewed and summarized the previously described cases of pediatric testicular vein thrombosis.

HWA: Hypoferritinemia without anemia a hidden hematology disorder.

INTRODUCTION: Anemia is a condition, in which the number of red blood cells (RBC) and the hemoglobin (Hb) is insufficient to meet the body's physiologic needs. Hypoferritinemia without anemia (HWA) could cause masked clinical manifestations as general weakness, easy fatigability, depressed mode, and hair loss but with normal complete blood count (CBC) parameters. HWA is deferent from iron deficiency anemia (IDA) because there is no reduction in the RBC, Hb, or any of the other parameters of the CBC. In HWA, the hypoferritinemia could be missed because serum ferritin not always requested by general practitioners. MATERIALS AND METHODS: A retrospective study conducted on 6993 patients of hypoferritinemia patients from our computerized database from 2010 to 2016. The parameters investigated to detect HWA disorder were serum ferritin, RBC, Hb, hematocrit, mean corpuscular volume, and mean corpuscular hemoglobin. RESULTS: HWA is found in 130 (0.018%) hypoferritinemia patients, in a ratio 1.7-1 females to male. CONCLUSION: HWA is a masked hematology disorder which needs new nomenclature to distinguish it from IDA because they are two deferent disorders although both of them have low serum ferritin. HWA is not a latent IDA because it could happen in patients whom will not get anemia as in polycythemia patients on recurrent veinesection treatement.

Long Standing Eculizumab Treatment without Anticoagulant Therapy in High-Risk Thrombogenic Paroxysmal Nocturnal Hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-orphan disease affecting all hematopoietic cell types. The abnormality of red blood cells in this disease predisposes to intravascular complement-mediated hemolysis. Eculizumab is an orphan drug used to treat this rare disease. Thrombosis is the key cause of death in PNH patients in about 40% to 67% of cases. We report the case of a woman presenting with PNH complicated with serious Budd-Chiari syndrome thrombosis and with a stent inserted in the portal vein. She refused to take any anticoagulant treatment since she commenced eculizumab 4 years before. No thrombotic events happened since that time. This case could add an extra benefit for eculizumab, which could be used as an anti-thromboembolic prophylactic agent in PNH, especially in patients with thrombocytopenia, where the use of anticoagulant agents is extremely hazardous. More randomized studies might establish the use of eculizumab without anticoagulants to avoid serious bleeding that could happen in thrombocytopenic PNH patients.

Successful nilotinib treatment in a child with chronic myeloid leukemia.

A 16-year-old female was diagnosed incedentally with chronic myeloid leukemia (CML) in the chronic phase. She showed complete remission after 3 months of nilotinib treatment. CML is a rare malignant neoplasm in pediatric age. It is characterized by a Philadelphia chromosome, which comes from a genetic translocation between chromosomes 9 and 22. This translocation results in an abnormal fusion called BCR-ABL oncogene which encodes a chimeric BCR-ABL protein. This protein is the underlying cause of CML. Nilotinib is a newly licensed drug for CML in adults. Structurally, it is similar to imatinib (the older tyrosine kinase inhibitor), but it is much more potent in inhibiting BCR-ABL due to its much increased affinity for its binding site. Specific guidelines for CML treatment in children have yet to be determined. In our patient, nilotinib was used as an off-label drug because it is not licensed for children. According to the pharmacokinetic response to drugs, children cannot be considered small adults irrespective of their weight. Off-label drug use based on evidence that it is the best treatment available is an important tool in the hands of expert treating physicians.

Severe thrombocytopenia in a patient with hepatitis C treated with eltrombopag from off-label drug use to on-label drug use: a case report.

INTRODUCTION: Off-label drug use refers to drug use beyond the specifications authorized for marketing. Eltrombopag is a new thrombopoietin receptor agonist which was used successfully in this critical case of thrombocytopenia associated with hepatitis C infection before it became an approved drug for such cases. CASE PRESENTATION: A 56-year-old Kuwaiti woman with hepatitis C virus infection was treated with pegylated interferon α-2a and ribavirin, laboratory test results prior to therapy were within normal values. After 4 weeks of that treatment, she developed neutropenia and severe thrombocytopenia. Her hepatitis C virus treatment was stopped for many years until eltrombopag was used as an off-label drug with an episode of severe thrombocytopenia. Her platelets count returned to normal level when triple therapy for hepatitis C virus was used successfully. CONCLUSIONS: An off-label drug should be used only when it is the best available drug, based on evidence from on-going multicenter trials. It could be life saving for some patients in critical situations. However, clinical use of eltrombopag later confirmed that it is a safe and effective drug for immune thrombocytopenic purpura or thrombocytopenia associated with hepatitis C virus infection.

Splenectomy complicated by sustained extreme thrombocytosis and extensive portosplenomesenteric vein thrombosis in pyrimidine 5'-nucleotidase deficiency.

Reactive and redistributional thrombocytosis is a well-known postsplenectomy occurrence .Usually it is transient and it rarely reaches extreme levels. We report a rare case of haemolytic anaemia where splenectomy was carried out following trauma to a massively enlarged spleen and was followed by extreme sustained thrombocytosis associated with extensive portal, splenic and mesenteric vein thrombosis despite standard antithrombotic prophylaxis.

Can lenalidomide play a role in the management of scleritis?

Lenalidomide is an immunomodulatory agent that was approved for the treatment of a monoclonal bone marrow disorders, myelodysplastic syndrome del(5q)(MDS del(5q)), in 2005; the drug was subsequently also approved for the treatment of refractory multiple myeloma, a bone marrow malignancy of the B-lymphocyte lineage. The purpose of this study is to report a case of MDS del(5q) in a female patient, which was most likely secondary to the immunosuppressive drugs that the patient was taking for scleritis. After lenalidomide treatment, the patient's haematological symptoms rapidly resolved and she became transfusion independent, with normal haemoglobin levels. This medication also helped control her dependence on high doses of oral prednisolone. The patient continued to receive treatment with low-dose lenalidomide, and her scleritis has been in long-term remission for 3 years. A larger prospective study can further define the role of lenalidomide in the management of scleritis.

Complete Remission of del(5q) Myelodysplastic Syndrome after 7 Days of Lenalidomide Therapy Gives an Alert!

Myelodysplastic syndrome (MDS) refers to a group of haematological, monoclonal disorders. A 50-year-old woman was diagnosed with MDS 5q deletion syndrome [del(5q)], becoming dependent on blood transfusion after long-term treatment with cytotoxic drugs for chronic scleritis. Lenalidomide therapy (10 mg/day) led to profound pancytopaenia, followed by recovery of her blood cell counts. A cytogenetic study, repeated 4 months after lenalidomide treatment, revealed complete remission after only 1 week of lenalidomide therapy. Lenalidomide was approved for low- and intermediate-1-risk MDS, where it normalises platelet counts and induces haematological and cytogenetic remission. This patient has remained transfusion independent for 3 years by continuing on a minimal maintenance dose of lenalidomide. Starting MDS patients on lenalidomide has to be done cautiously or with only 5 mg/day because of the potentially high sensitivity of the stem cells to this immunomodulatory agent in MDS patients.