Gurinder Sidhu

Findings of Multiple Myeloma in Afro-Caribbean Patients in the United States

Background Multiple myeloma (MM) is the second most common malignancy in the United States and has a higher incidence in the black and Afro-Caribbean population. There remain limited data on disease presentation and clinical characteristics in this patient group in the United States. The clinical profile of MM in this underrepresented patient group is described here. Methods This retrospective study was conducted at Kings County Hospital, an urban New York City hospital in a majority Afro-Caribbean neighborhood. Data from patients diagnosed with MM from 2000 through 2013 were collected from the institution’s tumor registry. Clinical and demographic characteristics of these patients were then analyzed. Results Patients with a diagnosis of MM were identified (N = 287). Data were available for 231 patients and of these, 97% self-identified as black. 55% were female, and there was a male-to-female ratio of 1:1.2. The mean age of female patients was 64 years; that of male patients was 63 years. Of the 231 patients, 81% had anemia, 68% had bone lesions, 47% had renal impairment, and 29% had hypercalcemia. Low levels of monoclonal protein were present in 27% of patients and 57% had disease of International Staging System stages I and II. Women had higher BMI than men. Conclusion The mean age of presentation of MM in Afro-Caribbean patients is similar to that in the standard population; however, unlike the general US population, there was a higher incidence in women; mean BMI of women also was higher than that of male patients. A sizeable percentage of Afro-Caribbean patients with MM presented with low levels of monoclonal protein in the presence of multiorgan involvement and damage, suggesting the need for early and aggressive diagnostic testing.

MP12: CRYOGLOBULINEMIA IN A PATIENT WITH CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)– A CASE REPORT AND REVIEW OF LITERATURE OF RENAL INVOLVEMENT IN CLL

Purpose of Study We report a case of early stage CLL that caused cryoglobulinemia-related glomerulonephritis (MPGN). This prompted a literature review to identify the incidence and causes of renal disease in patients with CLL. Methods Used Using a PUBMED search data of cases reported between 1990 and 2014, we selected cases with the following criteria: (1) Clinical and hematologic diagnosis of CLL (2) Evidence of renal insufficiency in association with CLL in the absence of other causes for proteinuria. Summary of Results Review of the literature revealed that 50 cases of CLL-related nephropathy have been reported, commonly with the nephrotic syndrome (MPGN). IHC staining of the renal biopsies of these patients revealed mostly monoclonal IgG kappa. Many of these were low stage CLL patients and had not been treated. In 68% the renal complications first developed years after the CLL was diagnosed. Most importantly, 65% of all patients nephropathies responded completely to anti-CLL therapy. Conclusions We report a rare manifestation of a secretory type of CLL with cryoglobulinemic vasculitis; leading to rapid renal deterioration needing dialysis. Renal damage can be a sequela of early-stage CLL due to its secretory phenotype. Timely recognition of kidney impairment and routine testing of serum or urine proteins in CLL patients is warranted to detect its early transition to a secretory phenotype. We believe that therapeutic intervention in early stage CLL may be effective in the preservation of renal function by removing the secretory phenotypic clone. Abstract MP12 Figure 1 IFE of cryoglobulins involving the kidney in our patient; predominantly IgG and kappa light chain (A&D) with weak expression of IgM lambda (B&D).

P11: ADULT T CELL LYMPHOMA (ATL) MASQUERADING AS FACIAL NERVE PARALYSIS

Purpose of Study ATL is a rare and aggressive peripheral T-cell neoplasm characterized by clonal human T-cell lymphotropic virus type-1 (HTLV-1) proviral DNA integration with host T lymphocytes.These patients commonly present with lymphadenopathy, skin rash, fever, fatigue or altered mental status. The prevalence of CNS disease varies from 3 to 50% and is always in the presence of systemic disease. Isolated cranial neuropathy as a presenting symptom has not been described in literature. Methods Used Retrospective chart review and review of literature. Summary of Results 49 year old Caribbean male presented with 2 month history of left sided headache, 5 weeks of right sided jaw numbness and pain which progressed to contralateral side. He was now unable to smile and had food falling from the side of his mouth. He denied fever, fatigue, night sweats, rash, weakness or abnormal lumps. He had normal mental status and good motor strength. Facial exam reveal bilateral upper and lower facial paralysis, left lateral rectus palsy and horizontal gaze diplopia. Rest of the physical exam was unremarkable. Labs revealed WBC of 6u2005k/mm3 with normal differential, HB 16.5 gm/dl and platelets 238u2005k/mm3. Complete metabolic profile and peripheral smear was normal. MRI Brain showed irregular, fusiform enhancement of left trigeminal nerve, bilateral facial and abducent nerves. CSF flow cytometry showed clonal CD4+ CD25+ T cell population. HTLV-1 serology was reactive. Left infraorbital nerve biopsy confirmed involvement with ATL. CT Chest/abdomen/pelvis did not reveal enlarged lymphadenopathy. He was started on treatment with EPOCH and twice weekly intrathecal methotrexate for 4 months with clearance of CNS fluid. His jaw pain and vision improved but facial nerve paralysis persisted. He developed local relapse four months after treatment and was treated with high dose methotrexate for 5 cycles. Ultimately his performance status deteriorated and he succumbed to the disease progression. Conclusions This case illustrates the unique presentation of this disease and gives an insight on one treatment approach. This patient achieved remission with our approach of aggressive chemotherapy with intrathecal methotrexate although the duration of remission was short lived.

Incidence of cytogenetic (CG) abnormalities in multiple myeloma (MM) in a minority population.

e18568 Background: Genomic aberrations are associated with MM survival and disease evolution. The combination of cytogenetics and Fluorescence in situ hybridization (FISH) reveals more anomalies than CG alone. The incidence of CG abnormalities in African American MM patients (AA) is not known.nnnMETHODSnRecords of patients with MM seen at Kings County Hospital and Downstate Medical Center from 2004 through 2010 were reviewed.nnnRESULTSnOf the 185 records reviewed, 181 (98%) patients had MM; 3 had plasma cell leukemia. Their age ranged from 36-89 years (median 66). There were 84 men (46%) and 97 women. CG analysis was done in 86 patients, 41 (48%) female and 45 (52%) male; of whom 82 were AA. CG was abnormal in 14 male and 6 female patients. Aneuploidy was present in 15 of 20 (75%); 11 of the 15 (73%) were hyperdiploid and 4 (26%) hypodiploid. Hyperdiploid chromosome number ranged from 49 to 67 (median 53). The most frequently aberrant chromosomes were 1, 4, 5 and 15. FISH was done in 42 patients for abnormal chromosomes 13, 14 and 17. One of 37 chromosomes 13 (2.6%), 3 of 28 (11%) chromosomes 14, and 1 of 14 (7.1%) chromosomes 17 displayed molecular abnormalities. In no case was more than one abnormality found. FISH on 28 patients with normal CG yielded only 1 (3.6%) abnormality.nnnCONCLUSIONSnAbnormal CG was not more frequent in this AA population than in MM patients from other ethnic groups, but was in male AA patients. Fewer molecular abnormalities were found in this population.

Incidence of cytogenetic (CG) abnormalities in acute myeloid leukemia (AML) in African American (AA) population.

6622 Background: CG characteristics play a critical role in outcome and therapy of AML. The incidence of normal CG in general population is estimated at 40%. The incidence of abnormal CG in AA population is not known. CG abnormalities help define the pathogenesis of AML subtypes and are prognostically significant; they occur in 40% of all AML cases, but their incidence in African Americans is not known.nnnMETHODSnRecords of adult patients with acute leukemia at Kings County Hospital and Downstate Medical Center from 2004 through 2010 were reviewed. The CG data were stratified into three prognostic groups: good; inv (16), t (8:21) and t (15:17); intermediate: normal CG, +8, t (9:11); poor: ≥ 3 CG abnormalities, including: -5, 5q-, -7, 7q-, inv (3), t (6:9), t (9:22).nnnRESULTSnOf the 52 patients with acute leukemia, 46 had AML; 29 were male and 17 female. Forty-three (93%) of these patients were AA. Ages ranged from 20-82 years (median 53), 15 (33%) having been over 65. Seven (15%) patients had a prior hematological malignancy (HM). CD34 was over-expressed in 23 of 46 (48%) patients with AML. CG data was available in 41 patients, 26 men and 15 women; and was normal in 13 (31%) and abnormal in 28 (69%). Of patients with de-novo AML and known CG; 21 (62%) were abnormal; and 13 (38%) were normal. The distribution of patients in risk stratified groups were: good: 8 (20%); intermediate: 19 (46%); and poor: 14 (34%). In patients with prior HM five (71%) had abnormal CG. The median age of patients with good, intermediate and poor risk CG was 57, 53 and 57 years respectively. Intermediate or poor risk AML occurred in 22 of 26 men (85%) and 11 of 15 women (73%).nnnCONCLUSIONSnOur data suggest that CG in AA AML patients are more often abnormal, and of intermediate or poor risk than in the general population.