Jameel Muzaffar

Successful Treatment of Bing-Neel Syndrome Using Intrathecal Chemotherapy and Systemic Combination Chemotherapy Followed by BEAM Auto-Transplant: A Case Report and Review of Literature

Neurological complications occur in approximately 25% ofpatients with WM. Although they are most often present as pe-ripheral neuropathy, hyperviscosity can produce central nervoussystem (CNS) changes that resolve promptly after plasma exchange.Rarely is the CNS directly involved as is the case in Bing-Neelsyndrome (BNS), which results from infiltration of pons, medulla,periventricular white matter, or leptomeningeal spaces by lympho-plasmacytoid cells.

Rituximab and intravenous immunoglobulin (IVIG) for the management of acquired factor VIII inhibitor in multiple myeloma: case report and review of literature

Acquired factor VIII inhibitor (AFI) is a rare disorder and is even more uncommon in multiple myeloma patients, with only five cases reported in literature. Solid and hematologic malignancies, autoimmune conditions, drugs, and infections are the conditions commonly associated with the development of this condition, with mucocutaneous bleeding being the most common presenting sign. Diagnosis is usually made with the laboratory finding of an elevated partial thromboplastin time aPTT that cannot be corrected by plasma mixing, and further confirmed by low factor VIII activity/antigen levels along with elevated factor VIII inhibitor levels using the Bethesda assay. Treatment is usually based on the clinical picture with factor VIII inhibitor bypass activity (FEIBA) and recombinant factor VIIa (rFVIIa) employed to control acute bleeding; steroids and cyclophosphamide to suppress the inhibitor with Rituximab, in combination with other immunosuppressants in cases not suitable for steroids, and finally wherever possible, to remove the offending drug or control the underlying pathology that might predispose to the development of this condition. This case report highlights the successful management of a myeloma patient who presented with life-threatening hemorrhagic pericardial effusion and hemarthrosis. The patient was treated with FEIBA to control the acute bleeding and then received Rituximab in combination with intravenous immunoglobulin to suppress the AFI.

Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis

Summary Background: Amyloidosis is a systemic disorder that results from the tissue deposition of various proteins with distinctive morphological characteristics. Conjunctival amyloidosis is a rare variant which is generally localized and not associated with systemic involvement. Case Report: We present here a case of 47-year-old female patient with right eyelid swelling that progressed over a 12 year period and eventually underwent surgery with pathology showing AL conjunctival amyloidosis. Unlike in most other reported cases of localized amyloidosis, she was noted to have amyloid deposition in the bone marrow and gastrointestinal tract upon extensive evaluation without any evidence of underlying plasma cell dyscrasia. She has been on observation without evidence of systemic progression or recurrence of conjunctival amyloid. Conclusions: Although it initially appeared that our case represented an isolated form of AL (kappa)-type conjunctival amyloidosis, systemic evaluation revealed trace amount of amyloid in the bone marrow and GI tract. It is feasible that upon very close scrutiny patients with seemingly localized AL amyloidosis may have trace amounts of amyloid involving other organs and based on experience from this single patient we believe that it is safe to observe such patients closely rather than pursue systemic therapy

High-Dose Chemotherapy and Autologous Stem Cell Transplantation for Multiple Myeloma in HIV-Positive Patients in the Highly Active Antiretroviral Therapy Era: The Myeloma Institute of Research and Therapy Experience

Introduction Non-Hodgkin lymphoma is the most common hematopoietic malignancy associated with acquired immune deficiency syndrome (AIDS). However multiple myeloma has also been reported a number f times in patients with human immunodeficiency virus (HIV) since 983 when the first case of a plasmacytoma was reported in a person with IV infection. Before the advent of highly active antiretroviral therapy (HAART) in 1996, the management of HIV-related hematopoietic malignancies was very difficult. High-dose chemotherapy and autologous stem cell transplantation (ASCT), which was standard therapy for he-

Preemptive intravenous immunoglobulin allows safe and timely administration of antineoplastic therapies in patients with multiple myeloma and parvovirus B19 disease

Parvovirus B19 (B19) disease is a rare cause of anemia in cancer patients and often goes unrecognized, causing delays in anticancer therapy.

459Clinical Characteristics and Outcomes of West Nile Neuroinvasive Disease in Immunocompromised Hosts: A case-Control Study

Disease in Immunocompromised Hosts: A case-Control Study Senu Apewokin, MD; Aasiya Matin, MD; Naveen Sanath Kumar, MD; Shebli Atrash, MD; Bakhous Aziz; Jameel Muzaffar; Vyjayanthi Ganga, MD; Monica Grazziutti, MD; Medicine, University Of Arkansas For Medical Sciences, Little Rock, AR; Myeloma Institute or Research and Therapy, UNIVERSTIY OF ARKANSAS FOR MEDICAL SCIENCES, LITTLE ROCK, AR; The Myeloma Institute for Research and Therapy/University of Arkansas for Medical Sciences, Little Rock, AR; Myeloma, UAMS myeloma institute., little rock, AR; Mirt, 4301 West Markham, little ROCK, AR

Waldenström's Macroglobulinemia Associated with Serum Amyloid A Protein Amyloidosis: Pitfalls in Diagnosis and Successful Treatment with Melphalan-Based Autologous Stem Cell Transplant

Waldenströms macroglobulinemia (WM) is increasingly being associated with amyloidosis particularly of the amyloid light-chain variety. We report on one of the few cases of WM associated with serum amyloid A protein (AA) amyloidosis. Autologous stem cell transplant (ASCT) is now being increasingly used for the treatment of amyloidosis, but most studies are small case series. Traditionally AA amyloid is associated with connective tissue disorders and periodic fever syndromes and has been treated by addressing the underlying condition. We present the first case of serum amyloid A being treated with melphalan-based ASCT to deal with the underlying WM and thereby control the amyloid, thus demonstrating the viability of this novel approach for the treatment of this disorder.

Bortezomib (BZB)-associated adverse events (AE) in patients with multiple myeloma (MM).

e18565 Background: In patients (pts) with MM, BZB therapy causes AE, some of which may be related to autonomic nervous system (ANS) dysfunction. METHODS We conducted a retrospective case control study of 5 pts, with pts serving as their own control. Each pt received 2 cycles of BZB-containing regimens (BZB with Dexamethasone and either Thalidomide or Lenalidomide). One of the two treatment courses used high-dose (HD) BZB (1.6 mg/m2, higher than the standard 1 to 1.3 mg/m2), while low-standard (LD) dose (0.7, 1.0 or 1.3 mg/m2) was applied during the other cycle. The medical records of these pts were reviewed, including for AE that are typically associated with ANS dysfunction like orthostatic hypotension, pyrexia (≥ 1020), severe diarrhea and severe constipation. Orthostatic hypotension was used as a measurable toxicity parameter with statistical comparison of its incidence (Pearson Chi Square test) between three groups: 1) pre-BZB therapy (control); 2) LD and 3) HD BZB cycles. RESULTS Cycles of LD BZB were well tolerated in all 5 patients in contrast to poor toleration in 4 of the 5 HD BZB cycles which required aggressive medical management for severe dizziness (1 pt) and hospitalization in 3 pts: 1) severe constipation and pyrexia(≥ 1020); 2) severe diarrhea, orthostatic hypotension and dizziness and 3) severe orthostatic hypotension. A specific etiology for these AE could not be identified despite a comprehensive work-up. The AE normalized by day 0 of the second cycle. Orthostatic hypotension was observed in 3 of 5 and 5 of 5 of LD and HD BZB cycles respectively versus 0 of 5 in the control group. A significant incidence of orthostatic hypotension was observed with HD BZB vs. control (α = 0.0079; α<0.05 is significant) and with both BZB groups combined vs. control (α= 0.0093). CONCLUSIONS HD BZB therapy is associated with otherwise unexplained, but transient orthostatic hypotension, pyrexia, and severe diarrhea and constipation. These findings are classically observed in ANS disorders, suggesting that some of BZB-related AE may be caused by transient ANS dysfunction.