A Tma Case Studies

Nurden AT, Pillois X, Wilcox, DA. Glanzmanns thrombasthenia: state of the art, and future directions. Semin Thromb Hemost. 2013; 39: 642-55

Myeloproliferative neoplasms (MPNs) are characterized by the proliferation of one or more cell types of the myeloid lineage, including erythroid, granulocytic, monocytic, or megakaryocytic lineages. These disorders are clonal and originate from a single pluripotent hemapoietic stem cell (Hubbard, 2011). MPNs have a slow onset and are mainly diagnosed in adults between 45 and 55 years of age. Clinical symptoms include anemia, weight loss, lack of energy, spleen enlargement, fever, and bruising (Hubbard, 2011).

This patient is mostly likely suffering from Post-transplant lymphoproliferative disorder, commonly called PTLD. Besides skin cancer, PTLD is one of the most common malignancies of post transplantation (LaCasce, 2006). Approximately 15% of transplant patients develop PTLD and it has a higher incidence amongst children than adults (DynaMed Plus, 2017) PTLD has a variety of presentations which lends to the difficulty and diversity of treatment options used for the disorder. The sites affected by PTLD and the incidence of the disorder depend its association with Epstein Barr virus and the transplant organ (LaCasce, 2006). The associated EBV infection is most commonly due to the donor graft and less commonly due to new environmental exposure

Unlikely, but the most common side effects and adverse reactions (incidence ≥ 2%) are hypersensitivity, bleeding, anemia, post-operative wound infection, thromboembolic events, hypotension, pyrexia, tachycardia and thrombocytopenia. For normal healthy adults, where bleeding is profuse, the dose of Thrombin-JMI is usually concentrations of 1,000 International Units per mL and dental extractions, skin grafting, etc. solutions containing approximately 100 International Units/mL are frequently used (Chuna, 2016).

Hemarthroses are responsible for roughly 75 percent of bleeding episodes in patients with severe hemophilia A degree (Roberts et al, 2010). Signs of hemarthroses include damage to capillaries located under synovial layer caused by mechanical trauma related to joints involvement in the regular daily

Allogeneic HSCT (hematopoietic stem cell transplantation) has been a path-breaking discovery in the field of hemato-oncology, proven to be a cure for many diseases like red-cell disorders and inborn errors. Technical and supportive care has resulted in survival rates that exceed 70% within the first two years after HSCT. However, long-term survivors carry a high burden of morbidity, including infections, treatment related organ toxicities, musculoskeletal disorders, endocrinopathies and immunological complications like Graft-versus-Host-Disease (GVHD). These usually affect multiple organs and tissues, and exhibit a variable clinical course, but in any case there should be an early recognition and the treatment has to be one of an aggressive

After careful review of the patient’s labs, WBC elevated and symptoms of low grade fever reflect that there is some sort of organism growth or inflammatory response in the body possible at the surgical site. The patient’s immature neutrophils(segs) elvateted indicative sign of inflammation or infection. Patients hemoglobin of 8.6 is considerably fair for a patient with chronic kidney disease. Low hemoglobin a indicative marker for severity of kidney disease (Leeder et al., 2006). The kidney is responsible for making erythropoietin a hormone that stimulate the bone marrow to make red blood cells, if the kidney is not working well we expect the RBC production to decrease( our text book). The patient elevated PT/INR is reflective on the positive fecal occult blood. Unfortunately Coumadin therapy most resume or continue because of her history of A-Fib. The sedimentation rate elevated is expected for history diagnosis of rheumatoid arthritis.

T.C. is a 30-year-old woman who 3 weeks ago underwent a vaginal hysterectomy and right salpingooophorectomy for abdominal pain and endometriosis. Postoperatively she experienced an intraabdominal hemorrhage, and her hematocrit (Hct) dropped from 40.5% to 21%. She was transfused with 3 units of packed RBCs (PRBCs). After discharge she continued to have abdominal pain, chills, and fever and was subsequently readmitted twice: once for treatment of postoperative infection and the second time for evacuation of a pelvic hematoma. Despite treatment, T.C. continued to have abdominal pain, chills, fever, and nausea and vomiting (N/V).

“Disseminated intravascular coagulation (DIC) is an acquired clinical syndrome characterized by widespread activation of coagulation, resulting in formation of fibrin clots in medium and small vessels throughout the body.” (Huether & McCance, 2012, p.527). There are many conditions associated with DIC, one of which is an acute leukemia. Diagnosis of DIC will begin with the patient’s medical history, a physical exam, and diagnostic test results. A cause for the DIC would also need to be determined.

A procedure known as transplantation consists of the transfer of cells, tissue or organ from one part of the body to another or from one person to another (National Cancer Institute, no date). Haemopoietic stem cells (HSCs) are unspecialized cells that have the ability to self-renew and the ability to differentiate into the HSCs lineage (Figure 1) for instance as white blood cells (WBCs), red blood cells (RBCs) and platelets. Treatment for a range of haematological disorders usually requires transfusion and transplantation (Knight, 2013). To obtain HSCs for transplantation there are three main sources. They are the bone marrow (BM), peripheral blood stem cells (PBSCs) and umbilical cord blood (UCB) (Smith and Wagner, 2010). A haploidentical donor is another source where the HLA match is not complete and there is a match of one haplotype. HSCs can be transplanted by an autologous, allogeneic and syngeneic donor (Hatzimichael and Tuthill, 2010). HSCs transplantation is carried out to eliminate the condition by myeloablation (prevent immune system attacking/rejecting new graft), to prevent the body being attacked by the new immune system derived from the new HSCs by graft versus host disease (GvHD) and to avoid rejection of the graft by immunosuppression (Buckland, 2017).

Blood and marrow stem cell transplantation is the preferred treatment for young patients who are otherwise in good

Terwey, T. H., Kim, T. D., & Arnold, R. (2009). Allogeneic hematopoietic stem cell transplantation for adult acute lymphocytic leukemia. Current Hematologic

It is important to impair or remove the T cells from the transplant donor tissue because if this doesn't happen graft vs host disease could occur. When both the recipient and tissue from the donor transplant are not histocompatible one or the other could reject each other. The T cells in the donor will most likely recognize the HLA antigens within the recipient to be foreign. When this occurs the graft will usually reject the host. This can occur through two forms, either chronic GvHD or acute GvHD. Chronic GvHD can happen when the HLA’s match however it takes a few months to years for GvHD to develop and often times results in an autoimmunity issue due to an abundance of cytokines being released. Acute GvHD generally occurs with bone marrow transplants a couple to several weeks after the initial transplant and the host becomes immunosuppressed as a result.

In most serious cases the blood is not able to reach the organs, resulting in enlargement of the liver, blood clots, stroke, and heart attacks. Thrombosis and Myelofibrosis are two other complications that are related to PV. George Krucik, MD, MBA on August 20, 2015 .

This is a 3-years-old African American male, who presented to C.S. Mott Children's Hospital Hematology/Oncology unit today from the hematology clinic to continue Interim Maintenance #1 today for day 15 to receive Vincristine and high dose of methotrexate. He was accompanied by both parents. We obtained the history from M.C., his parents and previous hospital reports. His primary is Pre-B Cell Acute Lymphoblastic Leukemia in 5/25/2016 on AALL1131 high risk protocol. He generally has been doing well except that he developed lower lip sores after last discharge as indicated by his mother. Parents applied Aquaphor and Vaseline which helps to reduce the sores. Lip sores did bleed occasionally, but only bother him when his mother was trying to apply