Acute ST-Segment Elevation Myocardial Infarction as Initial Presentation of Atypical Hemolytic-Uremic Syndrome.

A young woman presented with an acute ST-segment elevation myocardial infarction. Her clinical course was complicated by cardiogenic shock and acute renal failure. Work-up revealed thrombocytopenia and hemolytic anemia. A diagnosis of atypical hemolytic-uremic syndrome was made on the basis of clinical and pathological findings. (Level of Difficulty: Intermediate.).

Fatal refractory cardiac arrest as presentation of systemic amyloidosis.

Amyloidosis is a challenging diagnosis in the absence of clinical suspicion. The two patients reported here lacked any symptoms suggestive of amyloidosis. Instead, they presented with sudden, unexplained cardiac arrests that were persistent, treatment refractory, and ultimately fatal. Post-mortem examination unexpectedly revealed evidence of diffuse, systemic amyloidosis throughout multiple organ systems. We bring awareness to this unusual presentation of systemic amyloidosis that portends to poor outcome.

First Report of Severe Acute Graft-Versus-Host Disease After Allogeneic Stem Cell Transplant in a Patient With Myelodysplastic Syndrome Treated With Atezolizumab: Literature Review.

Checkpoint inhibitors have become a widely used and available immunotherapy option for treating a variety of malignancies, including hematological malignancies. Patients receiving these therapies may go on to receive a curative allogeneic hematopoietic stem cell transplant (allo-HSCT). This presents a clinical challenge as the safety and efficacy of HSCT is not well reported in this subset of patients and residual programmed death-ligand 1 inhibition could potentially enhance allogeneic T-cell responses, improving the graft-versus-tumor effect, but also increasing the incidence and severity of immune complications such as graft-versus-host disease (GVHD). Here, this report includes a detailed literature review summarizing all available data on HSCT outcomes in the setting of using checkpoint inhibitor therapy pre-transplant. Moreover, we report a case of acute GVHD after allo-HSCT in a patient with high-risk myelodysplastic syndrome who received prior atezolizumab therapy, highlighting the importance of further research into this specific population in order to improve transplant-related outcomes.

Bone Marrow Features in Patients With Acute Myeloid Leukemia Treated With Novel Targeted Isocitrate Dehydrogenase 1/2 Inhibitors.

This case report aimed to review the bone marrow features of patients with acute myeloid leukemia (AML) treated with isocitrate dehydrogenase 1/2 (IDH1/2) inhibitors. Five patients with AML treated with an IDH1/2 inhibitor were identified and retrospectively reviewed. We described the morphologic and immunophenotypic findings in the bone marrow, as well as ancillary study results. Two patients showed a hypercellular bone marrow with morphologic and immunophenotypic differentiation of blasts. The bone marrow of one patient displayed a hypoplastic phase. Four of the five patients demonstrated unusual morphologic and/or immunophenotypic populations, including basophilia with mild alterations on the myeloid blasts, a small subset of blasts with expression of T-cell markers not seen in the original immunophenotype, a cluster of differentiation 117 (CD117)-positive progenitor population with erythroid differentiation, and another population reminiscent of erythroid differentiation. Unusual morphologic and immunophenotypic populations can be seen in the bone marrows of patients treated with IDH1/2 inhibitors in the presence or absence of definite residual disease. The significance of these populations is uncertain, but further studies could be helpful to understand the meaning of these findings.

The role of FLT3 inhibitors as maintenance therapy following hematopoietic stem cell transplant.

Activating mutations in FLT3 in acute myeloid leukemia (AML) portend a poor prognosis, and targeting FLT3 with a tyrosine kinase inhibitor has been an area of intense research recently. Most FLT3 mutated AML patients undergo hematopoietic stem cell transplantation (HSCT) as standard of care but a significant proportion of patients relapse. Although the use of FLT3 inhibitors in the pre-HSCT perspective is more clearly defined, its use in the post-HSCT scenario, when most relapses occur, remains unclear. In this review, we comprehensively present the data on the recent and ongoing studies evaluating the role of various FLT3 inhibitors in AML with a particular focus in the post-HSCT setting.

A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disease primarily of children, characterized by a severe hyperinflammatory state. We describe a case of adult onset familial HLH with a novel exon 19, c.1607G>T (p.Arg536Leu) heterozygous mutation of the UNC13D gene in a 40-year-old woman who developed HLH during her first and second pregnancies, both episodes occurring during the first trimester. Our patient was treated successfully both times with HLH-94 protocol following spontaneous abortions and is currently in the process of getting a bone marrow transplant. We also discuss pregnancy as a potential trigger for late onset familial HLH.

A 63-Year-Old Woman With Neurofibromatosis Type 1 and Pulmonary Hypertension With Worsening Hypoxemia.

CASE PRESENTATION: A 63-year-old woman with a history of neurofibromatosis type-1 (NF-1) and pulmonary arterial hypertension (PAH) thought to be secondary to the NF-1 presented with a few weeks of worsening dyspnea on exertion. She took no medications other than sildenafil for her pulmonary hypertension (PH). She denied tobacco, alcohol, and illicit or anorectic drug use. She had previously worked as a waitress. Her mother and her brother had NF-1 but no PH or lung disease.

Sweet's syndrome associated with clonal hematopoiesis of indeterminate potential responsive to 5-azacitidine.

Sweet's syndrome (SS) is a rare condition characterized by the abrupt appearance of painful skin lesions due to neutrophilic dermal infiltration. Hematologic neoplasms, particularly acute myeloid leukemia (AML) and myelodysplastic syndromes (MDSs), have been commonly reported in association with SS. Clonal hematopoiesis of indeterminate potential (CHIP) is an emerging entity that is a precursor state to myeloid neoplasms. CHIP has not been previously associated with SS. We report the case of a 71-year-old man who presented with recurrent, painful edematous and erythematous papules and nodules for 18 months despite treatment with corticosteroids. He had normal blood counts, but a macrocytosis was noted (110 fl). Alternative causes of macrocytosis were ruled out. A skin biopsy confirmed a diagnosis of SS. Bone marrow biopsy specimen yielded a normal karyotype except for loss of the Y chromosome and equivocal morphologic findings. Polymerase chain reaction (PCR) and reverse transcription polymerase chain reaction (RT-PCR) of selected genes from the peripheral blood demonstrated a mixed lineage leukemia (MLL) partial tandem duplication (PTD) and sequence variant in CCAAT/enhancer binding protein alpha (CEBPA). These findings were consistent with a diagnosis of CHIP. The patient was treated with 5-azacitidine and achieved a complete remission of his skin lesions and was able to discontinue corticosteroids. To our knowledge, this is the first report of a patient with recurrent SS associated with CHIP. In addition to other myeloid neoplasms like AML and MDS, CHIP should be considered as a potential etiology in cases of recurrent SS. Treatment with a hypomethylating agents such as azacitidine could also serve as an alternative to systemic corticosteroid therapy.