Combined life-threatening internal organ bleeding and postpartum hemorrhage associated with acquired hemophilia A.

Key Clinical Message: Acquired hemophilia A (AHA) can present as life-threatening bleeding during the postpartum period. Prompt treatment allows patients with AHA to achieve complete remission and have normal subsequent pregnancies. Abstract: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the production of autoantibodies against factor VIII (FVIII). AHA can present with severe bleeding, especially in postpartum patient. We report a 38-year-old woman who presented in an emergency department with severe postpartum hemorrhage 2 weeks after cesarean section. Her investigation showed an isolated prolongation of partial thromboplastin time (PTT), low factor VIII assay and a factor VIII inhibitor test, resulting in abnormal Bethesda units which consistent with AHA. This case report highlights the importance of early diagnosis and treatment of AHA. With timely and appropriate management, most patients can achieve a good outcome.

Organomegalies as a predictive indicator of leukemia cutis in patients with acute myeloid leukemia.

BACKGROUND: Leukemia cutis (LC) is an extramedullary acute myeloid leukemia (AML) infiltrate. No previous study has described the clinical characteristics and outcomes of Thai patients diagnosed with AML with LC. MATERIALS AND METHODS: We conducted a 7-year retrospective case-control study on Thai AML patients at Siriraj Hospital from November 2013 to July 2020. Patients were divided into LC and non-LC groups. Initial clinical presentations and laboratory findings were examined to identify LC-associated factors. Overall survival (OS) and relapse-free survival (RFS) were assessed. Pathological tissues underwent re-evaluation to validate the LC diagnoses. RESULTS: The study included 159 patients in a 2:1 ratio (106 non-LC and 53 LC). The LC group had a mean ± SD age of 54.3 ± 15.5 years; females were predominant. Three-fifths of the LC patients had intermediate-risk cytogenetics; 20.4% had an adverse risk, and 10.2% had a favorable risk. Most were classified as AML-M4 and AML-M5. Leukemic nodules were the primary finding in 58.5% of the cases, mainly on the legs. In the multivariate analysis of predictive factors associated with LC, organomegalies, specifically hepatomegaly, and lymphadenopathy, remained significant factors associated with LC [OR 4.45 (95%CI 1.20, 16.50); p = 0.026 and OR 5.48 (95%CI 1.65, 18.20); p = 0.005], respectively. The LC group demonstrated a significantly reduced OS (log-rank test p = 0.002) (median OS of 8.6 months vs. 32.4 months). RFS was considerably lower in the LC group (log-rank test p = 0.001) (median duration of 10.3 months vs. 24.4 months in the non-LC). CONCLUSIONS: AML patients who developed LC tended to experience notably poorer prognoses. Therefore, it is imperative to consider aggressive treatment options for such individuals. The presence of organomegalies in AML patients serves as a strong predictor of the possible occurrence of LC when accompanied by skin lesions.

Revealing the Unanticipated: An Uncommon Case of Colorectal Adenocarcinoma Transitioning to Choriocarcinoma - A Case Report and Literature Review.

Choriocarcinomas are uncommon tumors, with non-gestational types occurring in both males and females. Primary choriocarcinoma of the colon is extremely rare. It presents significant diagnostic and therapeutic challenges due to its aggressive nature and poor prognosis, with no cure available, and a mean survival of 8 months. This case report describes a 48-year-old woman who presented with abdominal pain and an ovarian mass, initially suspected to be ovarian cancer. Further workup showed a primary tumor in the colon, with extension to the ovary and liver metastasis. The pathology findings confirmed the presence of colorectal adenocarcinoma with choriocarcinomatous differentiation, as indicated by immunohistochemistry. The patient initially responded to the cisplatin/etoposide regimen; however, she relapsed shortly after. The patient received additional treatments with pembrolizumab, paclitaxel, and olaparib, which resulted in partial remission. Despite challenges during treatment, such as suspected uveitis related to immune-checkpoint inhibitors and potential interference of antibodies with beta-human chorionic gonadotropin (ß-hCG) testing, the patient maintained a good performance status for over 1.5 years after being diagnosed. The case emphasizes the difficulties in treating choriocarcinomas, primarily because of their aggressiveness and the absence of standardized therapy. Our goal with this case is to draw multidisciplinary attention to this rare condition. Further studies are necessary to comprehend its clinical characteristics, prognosis factors, molecular markers, and treatment approaches. Such studies may be crucial in establishing targeted and personalized therapy. LEARNING POINTS: Primary choriocarcinoma of the colon is rare and often misdiagnosed due to its atypical presentation, complicating timely and accurate diagnosis.The aggressive nature of this tumor and lack of standardized therapy necessitates a multidisciplinary approach and personalized treatment plans, especially following relapse.Molecular profiling guided the use of immunotherapy, which showed potential but also presented challenges, highlighting the need for further research in treating this rare malignancy.

A Case of Subcutaneous Panniculitis-Like T-cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an HIV Patient.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs with hemophagocytic lymphohistocytosis, a life-threatening hyperinflammatory syndrome. The majority of SPTL cases express αß T-cell receptors (SPTL-AB) and have a favorable prognosis with oral immunosuppressive agents. We report a 37-year-old male patient with HIV infection who had a history of low-grade fever for one year, multiple tender subcutaneous nodules on both thighs, and cytopenia. He received several courses of antibiotics without significant improvement. A random skin biopsy showed lobular panniculitis and he was treated with steroids, but his fever recurred after steroid withdrawal. A second skin biopsy confirmed the diagnosis of SPTL. A bone marrow examination revealed hemophagocytic lymphohistiocytosis. He was successfully treated with cyclosporin A and prednisolone and achieved a complete response after one year of drug discontinuation. Panniculitis-like skin lesions have various etiologies and may present as a clinical mimic of lupus erythematosus panniculitis. The selection of an optimal site for skin biopsy is crucial to avoid erroneous diagnoses and adverse outcomes. We report a case of SPTL in an HIV-positive patient, which illustrates this diagnostic challenge.