Rosalind Catchatourian

Administration of ATRA to newly diagnosed patients with acute promyelocytic leukemia is delayed contributing to early hemorrhagic death

We hypothesized that the high early death rate (EDR) due to bleeding in acute promyelocytic leukemia (APL) is in part attributable to delays in all- trans retinoic acid (ATRA). We conducted a retrospective analysis of the timing of ATRA administration. 204 consecutive patients with newly diagnosed APL between 1992 and 2009 were identified. The EDR was 11%. 44% of early deaths occurred in the first week. Hemorrhage accounted for 61% of early deaths. ATRA was ordered the day APL was suspected in 31% of patients. Delays in ATRA administration led to increases in the percentage of early deaths from hemorrhage.

Localized insulin amyloidosis with use of concentrated insulin: a potential complication

Insulin‐derived amyloidosis is a rare form of amyloidosis composed of insulin fibrils. The pH and concentration of insulin are known to influence the conformational state of the insulin hormone, with an increasing concentration favouring a more complex conformation. Concentrated insulin delivers a large amount of insulin to a localized area, raising the possibility of inducing conformational changes, forming insulin fibrils and leading to localized insulin amyloidosis.

Polymorphic lymphoid proliferation presenting as ileocecal intussusception.

Dear Editor, Polymorphic lymphoid proliferation is a rare type of lymphoproliferative disorder (LPD) in immunodeficiency associated with human immunodeficiency virus (HIV) infection [1]. Here, we report a case of polymorphic lymphoid proliferation in a 30-year-old HIV+ African American female presenting as ileocecal intussusception. The patient presented to the emergency room with complaints of diarrhea, abdominal distention and pain for a week. CT scan revealed ileocecal intussusception. Laparotomy confirmed the ileocecal intussusception. Right hemicolectomy and ileostomy were performed, followed by ileostomy stump takedown procedure 3 months later. All bowel segments showed multiple small sessile polypoid lesions with unremarkable overlying mucosa. Histologically, the lesions consisted of sub-mucosal lymphoid aggregates with polymorphous populations of plasma cells, polyclonal lymphocytes with full range of maturation, and histiocytes. No cellular atypia and clonality or lymphoepithelial lesions were seen. Frequent lymphocytes were detected positive for Epstein–Barr virus (EBV) (Fig. 1). The cause of intussusception was due to EBV+ polymorphic lymphoid proliferation, which is HIV related. Meanwhile, the patient developed high-grade large (CD20+) B cell lymphoma involving the adenoid gland (Fig. 2) with extensive involvement of the bone marrow. She died shortly after, in less than 4 months of initial clinical presentation. LPD in immunodeficiency associated with HIV infection is of predominantly B cell origin. It consists of a heterogeneous spectrum of lesions ranging from polymorphic lymphoid proliferation, polymorphic B cell hyperplasia, to polymorphic and monomorphic B cell lymphomas. Most lesions are positive for EBV infection suggesting crucial pathogenetic roles. Among all lesions, polymorphic lymphoid proliferation is least common with less than 5% in ratio [1]. This is in drastic contrast to its counterpart, which occurs commonly in post-transplant lymphoproliferative disorder (PTLD) due to immunosuppression [1]. Intussusception caused by polymorphic lymphoid proliferation in this case is unusual due to the rarity of both entities. Unlike that in the pediatric scenario, the intussusception in adults is often associated with a pathological process, notably malignancy, as the lead point. It has been reported in association with enteric infection [2], reactive lymphoid hyperplasia [3], intestinal Kaposi’s sarcoma [4] and lymphomatous polyposis due to the T cell immunoblastic large cell lymphoma [5] in adults with HIV infection. Diagnosis of polymorphic lymphoid proliferation in LPD associated with HIV infection is often overlooked due to its seemingly benign histology [1]. According to the scant literatures on polymorphic lymphoid proliferation in HIV infection, morphologic presentation appears closely associated with molecular and genetic alterations [6, 7]. The attempt to define the lesion requires the consideration of Ann Hematol (2007) 86:453–454 DOI 10.1007/s00277-006-0241-y

Primary malignant myelomatous pleural effusion.

Primary malignant myelomatous pleural effusion (PMMPE) occurs in less than 1% of patients with multiple myeloma and is diagnosed either by visualization of plasma cells on cytology or by positive flow cytometry. The presence of immature plasma cells characterized by high nucleus to cytoplasm ratio, visible nucleolus and presence of Mott cells and Russell bodies are independent poor prognostic factors. The clinician should differentiate PMMPE from secondary pleural effusion as it is associated with a significantly worse prognosis and poor overall survival.

Health-related quality of life in underserved patients with hematologic malignancies.

238 Background: Patients diagnosed with hematologic malignancies have poor general health and the issues faced by vulnerable population segments further aggravate the situation. We aimed to explore the health-related quality of life (HRQoL) among underserved patients with hematologic malignancies. METHODS A cross-sectional study, including 95 patients diagnosed with hematologic malignancy, was conducted at Cook County Hospital, Chicago from Dec. 2014 to July 2015. European Organization for Research and Treatment of Cancer questionnaire was used to ascertain HRQoL. A standardized score (scale 0-100) was calculated for all variables and mean scores (MS) are reported. Higher score for HRQoL and functional scales and lower score on symptom scales indicated good health. Bivariate analysis, using t-test and ANOVA, was conducted to explore predictors of HRQoL. RESULTS The study included 48% Hispanics, 27% Afro-Americans, 14% Asians and 7% Caucasians. Median age was 53.8 years and 55% were males. Demographic analysis showed- 47% were fluent in english, 40% had insurance, 56% were US citizens, 14% were employed, 33% were high school graduates and 79% had good social support. Primary diagnosis were non-Hodgkin lymphoma (29.5%), AML (26%), ALL (17%), CML (9.5%), Hodgkin lymphoma (9.5%), CLL (4%) and multiple myeloma (4%). Median time since diagnosis was 2 years where 35% patients were in remission, 16% had relapsed disease and 58% were on chemotherapy. Overall HRQoL was poor (MS 62). Functional impairment was noted (MS 69) in all aspects including physical (MS 72), role (MS 66), emotional (MS 70), cognitive (MS 74) and social functioning (MS 65). Financial difficulties (MS 54), insomnia (MS 35), pain (MS 32), dyspnea (MS 24), poor appetite (MS 24), constipation (MS 23), fatigue (MS 17) and diarrhea (MS 14) were reported (symptom scale MS 29). Age, gender, socio-demographics, cancer type and treatment status did not significantly affect HRQoL, functional and symptom scores. Information regarding the disease was poor (MS 41). CONCLUSIONS Poor HRQoL amongst underserved patients was observed which should be addressed. Our findings warrant large-scale studies to explore resources amongst the urban underserved blood cancer patients.

Hypotension associated with advanced Hodgkin lymphoma

Hypotension is an extremely rare manifestation of Hodgkin lymphoma. We report the case of a patient who presented with new onset hypotension and was diagnosed with urosepsis and septic shock requiring pressor support for maintaining his blood pressure. computed tomography (CT) scan of abdomen showed liver lesions, which were new on comparison with a CT abdomen done 3 weeks back. Biopsy of the liver lesions and subsequently a bone marrow biopsy showed large atypical Reed-Sternberg cells, positive for CD15 and CD 30 and negative for CD45, CD3 and CD20 on immuno-histochemical staining, hence establishing the diagnosis of Hodgkin lymphoma. The mechanism involved in Hodgkin lymphoma causing hypotension remains anecdotal, but since it is mostly seen in patients with advanced Hodgkin lymphoma, it is hypothetically related to a complex interaction between cytokines and mediators of vasodilatation. Here we review relevant literature pertaining to presentation and pathogenesis of this elusive and rare association.

Frequency distribution of hepatitis C infection among patients with B-cell non-Hodgkin lymphoma in underserved minority population.

e18536 Background: The role of hepatitis C Virus (HCV) infection in the pathogenesis of non-Hodgkin lymphoma is not well clarified. Several studies show a variation (0% to 37%) in the prevalence of HCV infection in B-cell Non-Hodgkin Lymphoma (B-NHL) by geographical location. Mucosal-associated lymphoid tissue lymphoma (MALT) and diffuse large B-cell lymphoma (DLBCL) were reported to have the highest prevalence of HCV infection in subtype specific analysis of B-NHL in previous studies. METHODS We retrospectively studied 112 patients with histological diagnosis of B-NHL confirmed by flow cytometry and immunohistochemistry, consecutively seen from 2002 to 2007.Their clinical features and outcomes were evaluated. RESULTS Histopatholgy in patients with HCV was as follows: 12(67%) DLBCL, 3(16%) marginal zone lymphoma (MZL), 3(16%) Burkitt lymphoma (BL). 14(78%) patients had Stage III or IV disease, 11 (60%) had an IPI of ≥3, and 4(22%) had disease relapse. Regarding AA patients with HCV and B-NHL, the median age at diagnosis was 50 years, 6(60%) were males and 4 (40%) females. 8(80%) patients had DLBCL, 1(10%) had BL, and 1(10%) had MZL. 9 out of 10 had aggressive disease (DLBCL and BL). CONCLUSIONS The prevalence of HCV infection in patients with B-NHL we presente is higher than previously reported in some U.S. STUDIES 83% of patients with B-NHL / HCV infection, and 90% of AA with B-NHL/HCV, had aggressive disease, which is significantly higher than previously reported. This linkage should be further studied, and its relationship to other healthcare disparities in underserved populations should be evaluated. Of 112 patients, 18 (16%) were HCV positive, 17 (15%) were HIV positive and 6(5%) were hepatitis B (HBV) positive. Among HCV positive patients, the median age at diagnosis was 48 years; 12 (67%) were males and 6 (33%) females. 10 (55%) were African American (AA), 4 (22%) Caucasian, 3 (16%) Hispanic and 1 (5%) was Asian. 1 (5%) patient had coinfection with HBV and 2 (11%) had coinfection with HIV. [Table: see text] [Table: see text].

Presentation characteristics, risk profiles and long-term outcomes in a public health system, minority patient population with diffuse large B-cell lymphoma

8590 Background: Diffuse large B-cell lymphoma (DLBCL) is a common, aggressive form of non-Hodgkin lymphoma. We sought to characterize DLBCL in the minority patient (pt) population. Methods: 133 DL...