Plasmacytoma of the Nasolacrimal Duct Simulating Dacryocystitis: An Uncommon Presentation for Extramedullary Relapse of Multiple Myeloma

The most common site for localized forms of plasma cell neoplasms (extramedullary plasmacytoma; EMP) is the upper respiratory tract, including the oropharynx, nasal cavities, sinuses and larynx. A 50-year-old woman with a history of myeloma in complete remission after autologous stem cell transplantation complained of two weeks of epiphora of the left eye with subsequent diplopia, bloody nasal discharge and progressive swelling around the nasolacrimal sac. A solitary mass in the left sinonasal area, extending to the nasolacrimal duct (NLD) was detected on MRI, whose histopathological examination was consistent with plasmacytoma. Further clinical investigation ruled out multiple myeloma (MM). The patient underwent debulking surgery and adjuvant chemotherapy followed by local radiotherapy in an attempt to achieve complete response. Despite being a rare entity, EMP of the NLD should be considered in the differential diagnosis of epiphora and dacryocystitis. To our knowledge, this is the first case of a plasmacytoma of the NLD presenting as isolated extramedullary relapse of MM. The follow-up in EMPs should include appropriate imaging studies, a systemic workup to rule out MM

ERYTHROCYTE SEDIMENTATION RATE

Inflammatory responses to tissue injury (the acute phase response) include alteration in serum protein concentration, especially increases in fibrinogen, serum amyloid A protein and C-reactive protein, and decrease in albumin. The changes occur in acute infection, during active phases of chronic inflammation and following injury. Erythrocyte sedimentation rate (ESR) is one of the methods which may indicate ongoing acute phase responses and may determine the severity and extent of the inflammation. ESR may also indicate some types of cancer. ESR may correlate with the disease activity. The principle of the test is that red cells are falling in plasma to the bottom in a straight, vertical positioned anticoagulated venous blood tube with a rate due to the difference in specific gravity between red cells and plasma. The result is the height of the clear plasma above upper limit of the column of sedimenting cells and expressed as ESR= X mm in 1 h. The sedimentation rate is influenced mainly by the extent to which the red cells form roulox, which sediment more rapidly than single cells. Other factors, which affect sedimentation include the ratio of red cells to plasma and some changes related with tube, and otherwise. The ESR is influenced by age, stage of the menstrual cycle and drugs. The ESR is higher in women than in men. It is especially low in polycytaemia, hypofibrinogenemia and congestive heart failure, and when there are abnormalities of the red cells such as poikilocytosis, spherocytosis or sickle cell. Although a normal ESR cannot be taken to exclude the presence of organic disease, the fact remains that the vast majority of acute or chronic infections and most neoplastic and degenerative diseases are associated with changes in the plasma proteins, which lead to an acceleration of sedimentation

The use of emergency apheresis in the management of plasma cell disorders

Hyperviscosity syndrome (HVS) develops most commonly in Waldenstrtim's macroglobulinemia (WM) and multiple myeloma (MM). Plasmapheresis is the immediate therapy and very effective at relieving symptoms by removing paraprotein. The most commonly used replacement fluid is 4%-5% human albumin in physiologic saline. FFP may be used in patients with coagulation abnormalities. Plasmapheresis should be continued until acute symptoms abate. Hyperviscosity impairs the circulation in the retina and causes hemorrhages around the small retinal vessels. Early diagnosis and urgent plasmapheresis may reduce blindness caused by retinal hemorrhages and/or retinal detachment. In HCV related mixed cryoglobulinemias, plasmapheresis is indicated if rapidly evolving life-threatening disease with immunosuppressive agent exists. In non-infectious mixed cryoglobulinemia plasmapheresis is indicated when the disease manifestations are severe, as a second line option. In WM patients with hyperviscosity symptoms and IgM > 4 g/dL, preemptive plasmapheresis is recommended to prevent an IgM flare with rituximab. Certain IgG/A MGUS-associated neuropathy patients may benefit from plasmapheresis. For cast nephropathy (suspected or biopsy proven), plasmapheresis is recommended when the sFLC >= 500 mg/I and as early as possible (<1 month with kidney injury). Theoretically, extracorporeal removal alone, without efficient tumor killing, could not reduce sFLC due to high production by the tumor mass and rapid rebound between compartments. (C) 2018 Published by Elsevie

CYTOMEGALOVIRUS INFECTION AFTER HEMATOPOIETIC STEM CELL TRANSPLANTATION: THE CHANGE IN CLINICAL PICTURE, DIAGNOSIS, AND TREATMENT MODALITY

Cytomegalovirus (CMV) infection takes an important place as a major cause of morbidity and mortality after hematopoietic stem cell transplantation. There have been significant advances in understanding the pathogenesis of CMV, early detection of virus by modern laboratory technics, and conformable antiviral treatment. In this work, determination of high risk stem cell recipients for CMV infection and prophylactic (from the onset of engraftment until 100 days after transplantation) or preemptive (prevention of CMV disease after detection of CMV infection) treatment are reviewed in the context of important literature on this subject

Plasmapheresis/immunoadsorption in patients with pemphigus

Background and Design: Plasmapheresis and immunoadsorption, which is a more advanced method, are among the treatment options that induce a rapid remission in severe and resistant pemphigus. The aim of this study was to evaluate the efficacy and safety of plasmapheresis and immunoadsorption therapies via the clinical experience gained from our patients with pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP)

Plasmapheresis/immunoadsorption in patients with pemphigus

Background and Design: Plasmapheresis and immunoadsorption, which is a more advanced method, are among the treatment options that induce a rapid remission in severe and resistant pemphigus. The aim of this study was to evaluate the efficacy and safety of plasmapheresis and immunoadsorption therapies via the clinical experience gained from our patients with pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP)