Gastric adenocarcinoma with bone marrow carcinomatosis complicated with cancer related thrombotic microangiopathy: A case report (original) (raw)
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Gastric Adenocarcinoma Presenting as Thrombotic Microangiopathy in a 14-year-old Girl
Journal of Pediatric Hematology/Oncology, 2011
Gastric adenocarcinoma is rare in childhood and often presents with disseminated malignancy at diagnosis due to aspecific symptoms leading to delay in diagnosis. A familial predisposition for gastrointestinal cancer is suggested for the development of this early-onset adenocarcinoma. We report the case of a 14-year-old girl with a familial history of colorectal, liver, and breast cancers affected by metastatic gastric adenocarcinoma, who first presented with thrombotic microangiopathy. Thrombotic microangiopathy as first clinical presentation of metastatic gastric cancer is an exceptional event in childhood and represents a challenge for pediatricians. Gastric adenocarcinoma should be suspected in young patients with a significant familial history and also in the absence of initial specific signs, so as to provide correct diagnosis and appropriate treatment.
Thrombotic microangiopathy in cancer
Thrombosis Research
Thrombotic microangiopathy (TMA) is a rare but often devastating complication of cancer and cancer treatment. The syndrome is defined by thrombocytopenia (i.e., a platelet count of < 150,000/mL blood, or a reduction in platelet count by > 30% from baseline), evidence of microangiopathic hemolytic anemia (MAHA), and evidence of organ damage. There are nine recognized categories TMA including thrombotic thrombocytopenic purpura (TTP) hereditary and acquired deficiency of the von Willebrand cleaving protease, ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), congenital and acquired atypical hemolytic uremic syndrome (aHUS), Shiga toxin-related hemolytic uremic syndrome (STEC-HUS), metabolic causes due to abnormalities both congenital and acquired defects in the cobalamin pathway, coagulation disorders such as disseminated intravascular coagulation (DIC), drug-induced TMA due to direct toxic effects as well as immune-mediated endothelial damage, cancer, and chemotherapeutic treatment. 1,2 TMA has also been seen in clinical settings of inflammatory autoimmune disorders, including systemic lupus erythematosus and rheumatoid arthritis, the glomerulopathies, and bone marrow or solid organ transplantation. The presentations of TMA disorders may be indistinguishable clinically. Although the pathophysiology of the disorders varies, all are characterized by endothelial damage resulting in microvascular ischemia. 1,2 This review will discuss, in detail, TMA uniquely associated with cancer and cancer treatment. A simplified summary of causes, pathology, and treatment of cancer-associated TMAs can be found in â–ºTable 1. Tumor-Associated Thrombotic Microangiopathy Almost 50 years ago, TMA was first described in gastric cancer patients. Cancer is known to be associated with both macroand microvascular thrombosis. 1 TMA was reported with mucinous gastric cancers from as early as the 1970s. 3-5 Cobalamine deficiency, which can also cause a TMA-like syndrome by itself, can occur in gastric cancer and may contribute to the development of TMA in that setting. 6 In most cases, TMA is associated with advanced cancer. It has been identified in patients with metastatic gastric, ovarian cancer, prostate, lung cancer, urothelial cancers, lymphomas, and myeloproliferative neoplasm and acute myeloid leukemia. 7-10 Tumor embolization to the small vessels in the lungs, particularly from gastric, urothelial, and lung cancers, is associated with pulmonary tumor thrombotic microangiopathy (PTTM). 7-12 In one study, it occurred in 21/690 consecutive autopsies and is reported to occur in 0.3 to 3.3% of autopsies. This syndrome is associated with acute pulmonary decompensation, characterized by worsening dyspnea, and pulmonary hypertension. In addition to hypoxemia, the chest X-ray findings show ground glass opacities, without an infectious etiology. Pathologic findings include small vessel, pulmonary arteriolar occlusion with intimal proliferation, and fibrin deposition in the thrombi. 9,10 There is evidence of increased pulmonary vascular resistance. 7,11 The syndrome is rapidly fatal. 9,10 Pathologic features of tumor-induced TMA include vessel wall thickening at the arteriolar-capillary junction, with
Journal of Medicine, 2019
Microangiopathic Hemolytic Anemia (MAHA) is a hematological condition which is very rare for the primary presentation of a gastric adenocarcinoma with bone marrow metastases. When it emerges as initial findings in a previously undetected case of malignancy, the diagnosis is often missed and results in inappropriate management. Carcinoma stomach associated with MAHA is generally having fulminant course. This is a case report of a 30-year-old male who presented with widespread bone marrow infiltration along with Coomb's negative haemolytic anemia, thrombocytopenia and schistocytes in peripheral blood typical of MAHA. The combination of MAHA and bone marrow infiltration in gastric adenocarcinoma is a very rare entity. When the cause of progressive MAHA is unknown, the possibility of cancer-associated MAHA must be excluded by performing additional tumor workup, including the detection of tumor markers, Endoscopy of upper GIT, colonoscopy, bone marrow examinations, and PET-CT or bone scans.
Pulmonary Tumor Thrombotic Microangiopathy
Clinical Nuclear Medicine, 2009
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare clinical entity where tumor cell embolisms in pulmonary circulation induce thrombotic microangiopathy (TMA), respiratory failure, and subacute cor pulmonale. We describe 3 cases of PTTM that presented as the initial manifestation of metastatic gastric adenocarcinoma with TMA and pulmonary infiltrates. All 3 cases had similar clinical and laboratory features, which included moderate thrombocytopenia without renal failure, hemolysis with extremely high serum lactate dehydrogenase levels, leukoerythroblastosis in peripheral blood smear, altered coagulation tests, lymphadenopathies, and interstitial pulmonary infiltrates. All patients died within 2 weeks of diagnosis. Two cases were initially misdiagnosed as idiopathic thrombotic thrombocytopenic purpura and treated with plasma exchange with no response. One patient had bone marrow infiltration by malignant cells. Autopsies revealed PTTM associated with gastric disseminated adenocarcinoma (signet-ring cell type in 2 patients and poorly differentiated type in 1). PTTM should be considered in the differential diagnosis of patients with fulminant microangiopathic hemolytic anemia, such as atypical thrombotic thrombocytopenic purpura, mainly those with pulmonary infiltrates, disseminated intravascular coagulation, or Trousseau syndrome.
Pulmonary tumor thrombotic microangiopathy: report of 3 cases and review of the literature
Medicine, 2014
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare clinical entity where tumor cell embolisms in pulmonary circulation induce thrombotic microangiopathy (TMA), respiratory failure, and subacute cor pulmonale.We describe 3 cases of PTTM that presented as the initial manifestation of metastatic gastric adenocarcinoma with TMA and pulmonary infiltrates.All 3 cases had similar clinical and laboratory features, which included moderate thrombocytopenia without renal failure, hemolysis with extremely high serum lactate dehydrogenase levels, leukoerythroblastosis in peripheral blood smear, altered coagulation tests, lymphadenopathies, and interstitial pulmonary infiltrates. All patients died within 2 weeks of diagnosis. Two cases were initially misdiagnosed as idiopathic thrombotic thrombocytopenic purpura and treated with plasma exchange with no response. One patient had bone marrow infiltration by malignant cells. Autopsies revealed PTTM associated with gastric disseminated adeno...
Pulmonary Tumor Thrombotic Microangiopathy: Clinical, Radiologic, and Histologic Correlation
Journal of clinical imaging science, 2015
Pulmonary tumor thrombotic microangiopathy (PTTM) is a clinicopathologic disease entity in which the tumor cells embolize to the pulmonary vasculature leading to a series of maladaptive reactions including the activation of coagulation and fibrocellular intimal thickening. The resultant stenosis of blood vessels leads to pulmonary hypertension and eventual death from cor pulmonale. In this report, we present a case of PTTM presenting as the initial manifestation of metastatic gastric carcinoma in a young man. Although unusual in its occurrence as the initial manifestation of gastric carcinoma, the case is illustrative in its clinical, radiological and histological presentation.
Supportive Care in Cancer
Thrombotic microangiopathy (TMA) is a syndrome that encompasses a group of disorders defined by the presence of endothelial damage leading to abnormal activation of coagulation, microangiopathic hemolytic anemia and thrombocytopenia, occlusive (micro)vascular dysfunction, and organ damage. TMA may occur in patients with malignancy as a manifestation of cancer-related coagulopathy itself or tumor-induced TMA (Ti-TMA) as a paraneoplastic uncommon manifestation of Trousseau syndrome. TMA can also be triggered by other overlapping conditions such as infections or more frequently as an adverse effect of anticancer drugs (drug-induced TMA or Di-TMA) due to direct dose-dependent toxicity or a drugdependent antibody reaction. The clinical spectrum of TMA may vary widely from asymptomatic abnormal laboratory tests to acute severe potentially life-threatening forms due to massive microvascular occlusion. While TMA is a rare condition, its incidence may progressively increase within the context of the great development of anticancer drugs and the emerging scenarios in supportive care in cancer. The objective of the present narrative review is to provide a general perspective of the main causes, the key work-up clues that allow clinicians to diagnose and manage TMA in patients with solid tumors who develop anemia and thrombocytopenia due to frequent overlapping causes.
A report of disseminated adenocarcinoma presenting as thrombotic thrombocytopenic purpura
Hematology Reports, 2011
Thrombotic microangiopathies (TMAs) represent a heterogeneous group of diseases characterized by a microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. TMAs encompass thrombotic thrombocytopenic purpura (TTP), typically characterized by fever, central nervous system manifestations and hemolytic uremic syndrome (HUS), in which renal failure is the prominent abnormality. In patients with cancer TMAs may be related to various antineoplastic drugs or to the malignant disease itself. The reported series of patients with TMAs directly related to cancer are usually heterogeneous, retrospective, and encompass patients with hematologic malignancies with solid tumors or receiving chemotherapy, each of which may have distinct presentations and pathophysiological mechanisms. Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP) Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature. We present a case of a 34-year-old man with TTP syndrome secondary to metastatic small-bowel adenocarcinoma. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.
Cancer, 1981
Microangiopathic hemolytic anemia and thrombocytopenia secondary to disseminated intravascular coagulation is a well-described complication of widely metastatic carcinoma. The authors report four cases of gastric carcinoma, one case of colon cancer, and one case of adenocarcinoma of unknown primary in which the patient developed a syndrome analagous to thrombotic thrombocytopenic purpura, consisting of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure without definite evidence of disseminated intravascular coagulation. In contrast to previous reports, postmortem examination in three of the cases revealed no recurrence or only microscopic foci of residual tumor. In the remaining three, there was clinical and pathologic evidence of grossly disseminated carcinoma. Also in contrast to previous cases, all patients evidenced azotemia and proteinuria at the onset of the syndrome and ultimately uremia was a contributing cause of death. Coagulation profiles showed prolonged thrombin times and elevated fibrin degradation products in four instances and did not distinguish the patients with grossly metastatic disease from those with no tumor or only microscopic residua. Circulating immune complexes containing carcinoembryonic antigen were found in the patient with metastatic colon carcinoma. The syndrohe was clinically identical whether or not grossly metastatic tumor was present, and it should not be attributed to advanced disease without definite clinical or pathologic evidence of a recurrence.