Elizabeth Guevara

A rare case of intracerebral hemorrhage complicating heparin-induced thrombocytopenia with thrombosis: a clinical dilemma ameliorated by novel use of plasmapheresis

We report a case of heparin-induced thrombocytopenia with thrombosis type 2 (HITT 2) that was first complicated by intracerebral hemorrhage (ICH) and later by deep venous thrombosis (DVT). HITT 2 was initially managed conventionally with argatroban, which was stopped when ICH was discovered. The size of ICH increased despite attempts to increase platelet count by platelet transfusions. At this point of the clinical dilemma, plasma exchange was utilized effectively to recover the platelet count and deter ICH progression. The clinical course was later complicated by DVT, for which fondaparinux was given. This case represents a rare clinical scenario of HITT 2 resulting in progressive ICH that excluded the use of antithrombotic agents as part of HITT therapy. We believe that the use of plasmapheresis as a salvage procedure in such situations is effective and life-saving. Physicians should be aware of plasmapheresis as a therapeutic option in HITT 2 in cases in which anticoagulation is contraindicated.

Leptomeningeal involvement in a patient with splenic lymphoma with villous lymphocytes

Splenic lymphoma with villous lymphocytes (SLVL) is an indolent hematological malignancy. Persistent lymphocytosis and splenomegaly usually last for years before patients develop infectious complications. Organ involvement other than spleen and bone marrow is rare in SLVL. We report a case of SLVL-related meningitis occurring in a patient presenting with altered mental status and seizures. CNS involvement was documented by an MRI of the head and by the presence of atypical lymphocytes in the cerebrospinal fluid (CSF). Morphologic examination and immunophenotypic analyses were conducted to determine the nature of atypical lymphocytes in the peripheral blood, spleen, bone marrow and CSF. The patient improved after treatment with a combination of radiation and chemotherapy.

Benefit and risk of primary thromboprophylaxis in ambulatory patients with advanced pancreatic cancer receiving chemotherapy: A systematic review and meta-analysis of randomized controlled trials

As vascular thromboembolism (VTE) is a leading cause of death in cancer patients, it has been postulated that primary thromboprophylaxis (PTP) in cancer patients might improve survival by reducing VTE occurrence. We performed a systemic review and meta-analysis of randomized controlled trials (RCTs) to investigate the benefit and risk of PTP with low-molecular-weight heparins (LMWHs) in ambulatory advanced pancreatic cancer (APC) patients receiving chemotherapy. We undertook a literature search using MEDLINE and EMBASE databases through May 2015. RCTs with reduction in symptomatic VTE as a primary or secondary endpoints were included. Mantel–Haenszel method was used to estimate the pooled event-based risk ratio as well as the pooled absolute risk difference with 95% confidence interval (CI). Seven hundred and thirty-eight APC patients were eligible for analysis. PTP lasted 3–6 months. The crude VTE incidence was 2.1 and 11.2% in LMWH and in control groups, respectively (risk ratio, 0.18; 95% CI, 0.083–0.39; P < 0.0001). The absolute risk difference in VTE was −0.092 (95% CI, −0.127 to −0.057; P < 0.0001), with an estimated number needed to treat of 11 patients to prevent one symptomatic VTE event. The pooled risk ratio for major bleeding was 1.25 (95% CI, 0.48–3.3, P = 0.65). Although these findings are encouraging to deploy PTP in APC patients receiving chemotherapy, uncertainties remain as to its survival benefit, optimal PTP duration, type and dose of LMWH, and costs of care. Therefore, adequately powered randomized phase III studies are warranted to address these questions.

A Young Man With Disseminated Intra-abdominal Masses

A man in his 20s presented with a 2-month history of vague, right upper-quadrant abdominal pain, with associated 5-kg weight loss, loss of appetite, and increasing abdominal distension. He reported a small mass in the right scrotum that was evaluated 5 years previously and was reassured to be a nonmalignant lesion. Physical examination was notable for a distended abdomen with a palpable left abdominal mass. Testicular examination revealed an approximately 1-cm firm right spermatic cord nodule. Laboratory tests showed lactate dehydrogenase levels of 818 U/L (to convert to μkat/L, multiply by 0.0167), with no significant β-human chorionic gonadotropin and α-fetoprotein levels. Computed tomography (CT) of the abdomen (Figure, A and B) revealed innumerable masses throughout the abdomen and pelvis, with the largest measuring 17.5 × 14 × 14 cm and a 3.9-cm enhancing lesion in the left hepatic lobe, and a small amount of ascites. Testicular sonogram disclosed a 1.5-cm right epididymal cyst. Percutaneous biopsy and histologic examination of the dominant left abdominal mass revealed neoplastic cells (Figure, C) that were positive for cytokeratin, desmin but negative for S100. Fluorescence in situ hybridization (FISH) was positive for EWS-WT1 rearrangement. Computed tomographic image of abdomen and pelvis A Computed tomographic image of abdomen B

Primary Neuroendocrine Carcinoma of the Breast Metastatic to the Bones, Which Chemotherapy?

The primary neuroendocrine carcinoma (NEC) of the breast is defined as immunohistochemical expression of neuroendocrine markers (chromogranin and synoptophysin) in more than 50% of the neoplastic cells according to World Health Organization (WHO) classification of tumors in 2003 (Tumours of the Breast and Female Genital Organs, 2003, Lyon: IARC Press). It accounts for less than 5% of all cancers arising from the breast (Tumours of the Breast and Female Genital Organs, 2003, Lyon, France: IARC Press). However, based on the study conducted by Wang et al., the primary NEC of breast comprises less than 0.1% of all mammary carcinomas (Frankf Z Pathol, 73, 1963, 24). Because of the rarity of the disease and absence of the prospective trials, there is no standard treatment for primary NEC of the breast. Herein, we report the case of a middle age woman with primary NEC with bone metastasis.

Lymphocytic Platelet Satellitism.

We read with interest the recent report of Indranil Chakrabarti on platelet satellitism around polymorphonuclear leucocytes [1]. Even though it is a rare event as noted by the author, over thirty cases of neutrophilic platelet satellitism have been reported in the literature. By contrast, platelet satellitism around lymphocytes has rarely been mentioned in the literature. Therefore, we would like to take this opportunity to report our recent finding of lymphocytic platelet satellitism. A 76-year-old Hispanic man was evaluated for mild relative monocytosis without any other hematologic abnormalities. Examination of the peripheral blood smear revealed platelets rosetting lymphocytes with some platelets seemingly within the cytoplasm of lymphocytes (emperipolesis, which refers to the presence of an intact cell within the cytoplasm of another cell) (Fig. 1). Neither platelet aggregates nor satellitism around neutrophils was seen (inset). Smears prepared from the finger-prick blood sample did not show platelet satellitism. His white blood cell count was 8.8 9 10/L, hemoglobin 13.6 g/dL, and platelet count 150 9 10/L. Because the finger-prick smear demonstrated clearance of platelet satellitism, we assumed that the phenomenon was solely related to the anticoagulant ethylenediaminetetraacetic acid (EDTA). Platelet count in heparin or sodium citrate container or antiplatelet antibodies were not performed. There have been previous reports of platelet satellitism around neoplastic lymphoid cells and large granular lymphocytes [2–4]. In our patient, small to medium-sized normal lymphocytes were involved in both platelet satellitism and possible emperipolesis. The underlying mechanism is likely related to EDTA, antiplatelet antibodies, and activation of platelets rich in thrombospondin or some other cytoadhesive platelet a-granule proteins.

An Extremely Rare Case of Advanced Metastatic Small Cell Neuroendocrine Carcinoma of Sinonasal Tract

Small cell neuroendocrine carcinoma (SNEC) is a rare form of malignancy. It mainly presents as bronchogenic neoplasm, and the extrapulmonary form accounts for only 0.1% to 0.4% of all cancers. These extrapulmonary tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gall bladder, head and neck, cervix, and skin. Primary SNEC of the sinonasal tract is extremely rare with only less than 100 cases reported in the literature. Because of extreme rarity and aggressiveness of the tumor, the management for this entity varies considerably mandating multimodality approach. In this paper, we report a patient presented with left-sided facial swelling, and the histopathologic examination confirmed primary SNEC of left sinonasal tract. The tumor involved multiple paranasal sinuses with invasion into the left orbit and left infratemporal fossa and metastasized to cervical lymph nodes and bone. The patient encountered devastating outcome in spite of optimal medical management and treatment with palliative chemotherapy highlighting the necessity for further research of primary SNEC of head and neck.

A case of thrombotic thrombocytopenic purpura in late pregnancy

TO THE EDITOR: Thrombotic thrombocytopenic purpura (TTP) was first described by Eli Moschowitz about 90 years ago [1]. TTP is caused by a severe deficiency in the protein A disintegrin and metalloprotease with thrombospondin 1 motifs 13 (ADAMTS13), a metalloprotease that cleaves ultra-large von Willebrand factor (vWF) multimers [2]. Deficiency in this enzyme causes the accumulation of large vWF multimers, which increase platelet adhesiveness and impair fibrinolytic activity with subsequent thrombotic occlusion of the microvasculature. TTP is a rare and potentially life-threatening disorder. The incidence rate of suspected TTP-hemolytic uremic syndrome (TTP-HUS) in the general population is 11 cases per 1,000,000 people [3], compared to the estimated incidence of one in 25,000 deliveries [4]. The clinical features of TTP may resemble those of the more common pregnancy complications, such as preeclampsia or of hemolytic anemia, elevated liver enzymes, and low platelet count (HELLP) syndrome. However, the management of TTP is completely different from that of preeclampsia/HELLP. Here, we present the case of a young woman who developed TTP in late gestation.

A predictable but life-threatening complication of hydroxyurea in a patient with sickle cell anaemia: an experience learned from a Jehovah's Witness.

It is well known that hydroxyurea can cause pancytopaenia secondary to bone marrow suppression, which is reversible with short-term discontinuation of the therapy. However, it is important to note that bone marrow suppressive effects caused by hydroxyurea could be easily potentiated in patients with sickle cell anaemia complicated by chronic kidney disease (CKD). We present a case of a Jehovahs Witness with sickle cell anaemia, who developed severe bone marrow suppression due to the combined effects of hydroxyurea and CKD, resulting in a prolonged recovery period after discontinuation of hydroxyurea.

Spinal cord infarct as a presentation of cholangiocarcinoma with metastases

It is well-known that malignancies, particularly pancreatic and brain cancers, often present as venous thromboembolism. However, stroke and angina attributable to arterial occlusion are relatively common presentations as well. We are reporting a patient, with treatment-naïve hepatitis C and multiple liver nodules, was admitted for deep vein thrombosis (DVT) and pulmonary embolism (PE). Subsequently, she developed an ascending paralysis due to spinal cord infarct (SCI) despite adequate anticoagulation. She also had an enlargement of left supraclavicular lymph node, which was confirmed histologically metastatic cholangiocarcinoma. To our best knowledge, this is the first literature report showing the association linking SCI to metastatic cholangiocarcinoma as a consequence of hypercoagulable state of malignancy.