Outcomes of Patients with Thrombocytopenia Evaluated at Hematology Subspecialty Clinics

BACKGROUND: Thrombocytopenia is a frequently encountered laboratory abnormality and a common reason for hematology referrals. Workup for thrombocytopenia is not standardized and frequently does not follow an evidence-based algorithm. We conducted a systematic analysis to evaluate the laboratory testing and outcomes of patients evaluated for thrombocytopenia at hematology clinics in a tertiary referral center between 2013 and 2016. PATIENT AND METHODS: We performed a comprehensive chart review for patients evaluated for thrombocytopenia during the study period. Patients were followed for 1 year from the initial hematology evaluation and assessed for the development of a hematologic malignancy, rheumatologic, or infectious diseases among other clinical outcomes. RESULTS: We evaluated 472 patients with a median (range) age of 61 (17-94) years. The majority (63.8%) had mild thrombocytopenia. Within 1 year of follow-up, 14 patients (3.0%) were diagnosed with a hematologic malignancy. A higher likelihood of developing a hematologic malignancy was noted in patients with concurrent leukopenia (hazard ratio [HR] 9.97, 95% confidence interval [CI] 3.28-30.32, p \u3c .01) and increasing age (HR per 10-year deciles 1.52, 95% CI 1.03-2.25, p = .03). In patients with asymptomatic isolated mild thrombocytopenia, laboratory testing did not reveal any significant positive findings and patients did not receive any new major diagnosis during the follow-up period. CONCLUSION: Our findings provide basis and call for development of an evidence-based algorithmic approach for evaluation of patients with thrombocytopenia, testing, and referrals. It also supports a conservative approach mainly driven by physical exam signs, symptoms, and other laboratory findings for patients with isolated mild thrombocytopenia

Isolated Ocular Manifestation of Relapsed Chronic Myelogenous Leukemia Presenting as Myeloid Blast Crisis in a Patient on Imatinib Therapy: A Case Report and Review of the Literature

Blast phase in chronic myelogenous leukemia (CML) has rarely been reported to involve extramedullary sites like skin, lymph nodes, and central nervous system. Clinical history, characteristic hematologic findings (elevated leukocyte counts, myelocytic predominance, and basophilia), and Philadelphia chromosome are of high diagnostic significance especially in isolated extramedullary presentations. We describe a unique case of CML relapse with blast phase involving the eye. A 66-year-old man with a known diagnosis of CML on imatinib and in molecular remission for 3 years presented with a painful blind eye. Histologic examination revealed diffuse involvement of choroid, iris, vitreous humor, and the optic nerve by blast cells. The blasts expressed CD34, aberrant TdT, and a myeloid phenotype (CD13, CD33, and CD117). Fluorescence in situ hybridization (FISH) of vitreous fluid detected BCR-ABL1 gene rearrangement. Additionally, trisomy 8 and gains of 9 and 22 were seen which were not present in the initial diagnostic marrow study 3 years ago. At relapse, the bone marrow, peripheral blood, and the cerebrospinal fluid were not involved by CML. Patient received induction chemotherapy and single dose prophylactic intrathecal methotrexate and was maintained on antityrosine kinase therapy and eventually underwent allogenic stem cell transplantation

A mysterious cause of chest pain.

LEARNING OBJECTIVE #1: Recognize the clinical features of primary cardiac tumors LEARNING OBJECTIVE #2: Manage chest pain by ruling out the most serious and fatal etiologies first CASE: A 42-year-old African American male with a past medical history significant for previously treated non-medullary thyroid carcinoma presented to our hospital with acute onset pleuritic chest pain and dyspnea on exertion. Initial workup revealed a slightly elevated Troponin-I along with a diffuse ST-segment elevation on electrocardiogram. Left sided heart catheterization with coronary angiogram did not show any obstructive lesions. No evidence of pulmonary embolism was seen on computed tomography (CT) angiogram. A transthoracic echocardiogram showed a large right atrial mass, which was further evaluated by a cardiac magnetic resonance imaging (MRI) and found to be involving the right atrial wall and pericardium with evidence of central necrosis on T2 signal. Patient underwent gross total resection of the mass, pathology showed areas of spindle cells and vascular spaces which stained positive for CD31 and CD34 indicating vascular differentiation consistent with angiosarcoma. Staging CT scan of the chest, abdomen and pelvis did not show evidence of distant metastasis. Patient received adjuvant chemotherapy with adriamycin and ifosfamide, postchemotherapy cardiac MRI showed a possible evidence of persistent disease for which he received external beam radiation therapy (EBRT) to the remnant mass. Patient has been stable since then with no evidence of active disease for 2 years. IMPACT: Chest pain is a common presenting complaint in many patients, while the etiology is benign in several occasions, the clinical approach would differ for each particular case depending on several factors which should be incorporated to formulate a specific diagnostic approach and subsequent therapy plan. Younger patients with chest pain should be investigated thoroughly after ruling out deadly cardiac, pulmonary or vascular conditions. DISCUSSION: Primary cardiac tumors are very rare; based upon data from22 large autopsy series the approximate frequency is 0.02%, corresponding to 200 tumors in 1 million autopsies. Malignant tumors constitute around one quarter of these with the majority being sarcomas. Cardiac angiosarcoma typically affects middle aged males and it usually involves the right atrium with pericardial, caval and tricuspid-valve invasion. Metastases are usually found at time of presentation in up to 89% of cases with lungs being the most common site. Presentation is variable depending on the site of involvement. It can be incidentally discovered or might cause symptoms secondary to embolization, direct invasion of adjacent structures or hemodynamic instability due to obstructing cardiac flow. The mainstay of treatment for cardiac tumors remains surgical resection, however chemotherapy and in some occasions radiation therapy are essential adjuvant modalities to achieve cure

A mysterious cause of chest pain.

LEARNING OBJECTIVE #1: Recognize the clinical features of primary cardiac tumors LEARNING OBJECTIVE #2: Manage chest pain by ruling out the most serious and fatal etiologies first CASE: A 42-year-old African American male with a past medical history significant for previously treated non-medullary thyroid carcinoma presented to our hospital with acute onset pleuritic chest pain and dyspnea on exertion. Initial workup revealed a slightly elevated Troponin-I along with a diffuse ST-segment elevation on electrocardiogram. Left sided heart catheterization with coronary angiogram did not show any obstructive lesions. No evidence of pulmonary embolism was seen on computed tomography (CT) angiogram. A transthoracic echocardiogram showed a large right atrial mass, which was further evaluated by a cardiac magnetic resonance imaging (MRI) and found to be involving the right atrial wall and pericardium with evidence of central necrosis on T2 signal. Patient underwent gross total resection of the mass, pathology showed areas of spindle cells and vascular spaces which stained positive for CD31 and CD34 indicating vascular differentiation consistent with angiosarcoma. Staging CT scan of the chest, abdomen and pelvis did not show evidence of distant metastasis. Patient received adjuvant chemotherapy with adriamycin and ifosfamide, postchemotherapy cardiac MRI showed a possible evidence of persistent disease for which he received external beam radiation therapy (EBRT) to the remnant mass. Patient has been stable since then with no evidence of active disease for 2 years. IMPACT: Chest pain is a common presenting complaint in many patients, while the etiology is benign in several occasions, the clinical approach would differ for each particular case depending on several factors which should be incorporated to formulate a specific diagnostic approach and subsequent therapy plan. Younger patients with chest pain should be investigated thoroughly after ruling out deadly cardiac, pulmonary or vascular conditions. DISCUSSION: Primary cardiac tumors are very rare; based upon data from22 large autopsy series the approximate frequency is 0.02%, corresponding to 200 tumors in 1 million autopsies. Malignant tumors constitute around one quarter of these with the majority being sarcomas. Cardiac angiosarcoma typically affects middle aged males and it usually involves the right atrium with pericardial, caval and tricuspid-valve invasion. Metastases are usually found at time of presentation in up to 89% of cases with lungs being the most common site. Presentation is variable depending on the site of involvement. It can be incidentally discovered or might cause symptoms secondary to embolization, direct invasion of adjacent structures or hemodynamic instability due to obstructing cardiac flow. The mainstay of treatment for cardiac tumors remains surgical resection, however chemotherapy and in some occasions radiation therapy are essential adjuvant modalities to achieve cure

Clinical impact of metformin in diabetic diffuse large B-cell lymphoma patients: a case-control study

Molecular studies have shown metformin to have a promising effect in lymphoma; however, there is lack of studies translating this effect into clinical settings. This was a case-control study to assess the clinical effect of metformin in diabetic diffuse large B-cell lymphoma (DLBCL) patients. Case subjects were diabetic on metformin with a new diagnosis of DLBCL. A total of 24 case subjects were identified, and for each case a control was matched. Outcomes of this study were to assess overall response rate, complete remission rate, progression free survival, and overall survival between the two groups. There was a significant increase in overall response rate, complete remission rate, and improved progression free survival in the metformin group compared to the control group, however, no significant overall survival difference was observed. Metformin use might be associated with an improved response rates and progression-free survival in diabetic DLBCL patients

P-selectin expression assay in a repeatedly serotonin-release assay-negative patient with heparin-induced thrombocytopenia

Heparin-induced thrombocytopenia (HIT) is an immune complication of heparin therapy caused by antibodies to complexes of platelet factor 4 (PF4) and heparin. Pathogenic antibodies to PF4/heparin bind and activate platelets to propagate a hypercoagulable state culminating in life-threatening thrombosis. The serotonin-release assay (SRA) is considered the gold-standard test to diagnose HIT. However, the sensitivity of the SRA was questioned with reported cases of clinical diagnosis of HIT and negative SRA. Herein, we present the utility of platelet factor 4-dependent P-selectin expression assay (PEA) in diagnosing HIT in a patient with thrombocytopenia and recurrent thrombosis who repeatedly tested negative with SRA