Valproic Acid Induced Thrombocytopenia and Dysmegakaryopoiesis in a Pediatric Patient

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S98-S98
Author(s):  
J Langlie ◽  
M Huberman ◽  
Y Akgun

Abstract Introduction/Objective Valproic acid is a branched short chain fatty acid derivative that is used primarily to treat epilepsy as well as mood disorders, certain types of headaches, and neuropathic pain. It is commonly prescribed in the pediatric population and has shown to be effective for refractory epilepsy with adequate seizure control. Serious side effects may be prominent if the medication is not kept at the therapeutic range. A wide variety of known hematologic problems can be encountered including but not limited to anemia, thrombocytopenia, and leukopenia. Methods/Case Report We present a case of a pediatric patient with a past medical history significant for history of seizure disorder and who presented to Jackson Memorial Hospital for intermittent fevers and multiple unexplained bruises for 3 weeks as well as fatigue and weakness.The patient was recently started on valproic acid. Complete blood count (CBC) was obtained and showed a platelet count of 23 x10(3)/mcL with WBC of 3.5 x10(3)/mcL and hemoglobin of 9.7 g/dL.Serum valproate concentration was critically high (154 mg/L). Trephine biopsy showed a normocellular marrow (60%) showing maturing trilineage hematopoiesis and scattered atypical megakaryopoiesis characterized by small forms that are seen in relatively loose interstitial clusters (Figure 1). The marrow aspirate smears were characterized by cellular spicules with dysmegakaryopoiesis including numerous small hypolobated forms with frequent forms showing separated nuclei (Figure 2, 3, and 4). Blasts did not appear increased, comprising overall 1% of marrow cellularity. Karyotype studies revealed a normal female karyotype, 46, XX. FISH studies using probes commonly detected in MDS were negative. Next generation sequencing was negative for AML specific mutations including GATA1 and GATA2 mutations. Results (if a Case Study enter NA) N/A Conclusion This case report highlights the significant hematologic adverse effects of valproic acid, specifically pancytopenia with dysmegakaryopoiesis, raising the clinical suspicion of potential myelodysplastic syndrome. Critically high level of valproic acid (154 mg/L) and normalization of CBC after the stoppage of the medication strongly suggests that valproic acid can cause severe bone marrow suppression and specific morphologic atypia in the megakaryocytic lineage thus introducing a potential diagnostic pitfall. Because the CBC returned to normal, bone marrow biopsy was not repeated.

Author(s):  
Brent D. Bauman ◽  
Maria Evasovich ◽  
Amanda Louiselle ◽  
Eugene Zheng ◽  
Kevin Goodwin ◽  
...  

AbstractBackground:Hyperparathyroidism (HPT) is a rare disease in the pediatric population, and optimal management may be unclear if it is due to an occult parathyroid adenoma. We present a case report of a pediatric patient with an occult, ectopic, supernumerary, parathyroid adenoma.Case presentation:A 13-year-old female who initially presented with anxiety was diagnosed with HPT. Preoperative imaging and bilateral neck exploration with four-gland biopsy were negative for any parathyroid adenoma. Postoperative MRI identified a thymic mass. She subsequently underwent video-assisted thoracoscopic thymectomy with resection of an intrathymic parathyroid adenoma.Conclusions:The diagnosis of pediatric HPT is increasing. Supernumerary or occult parathyroid adenomas are rare and add complexity to presurgical planning and management. Our case represents the rare occurrence of a pediatric ectopic supernumerary occult parathyroid adenoma treated with a two-stage approach utilizing multiple imaging studies. We provide a review of the pathology and propose an algorithmic approach to manage these complex patients.


Author(s):  
Lauren Hennein ◽  
Nailyn Rasool ◽  
Maanasa Indaram

AbstractAn arachnoid cyst causing a compressive oculomotor nerve palsy is rare in the pediatric population. We describe a case of an acquired, partial oculomotor nerve palsy in a 3-year-old boy caused by an arachnoid cyst in the left crural cistern with associated amblyopia. The patient's amblyopia was aggressively treated, and he underwent cyst fenestration. Two months postoperatively, he continued to demonstrate a partial oculomotor palsy with improved visual acuity and recurrence of the cyst. This case demonstrates that cyst fenestration may not always resolve these paretic effects, cysts may recur after fenestration, and amblyopia must be treated in this setting.


Author(s):  
Dr. Vartika Sachdeva ◽  
Dr. Mansi Kala ◽  
Dr. Sushil Kumar Shukla ◽  
Dr. Anuradha Kusum ◽  
Dr. Kunal Das

Hairy cell leukemia is a chronic B cell lymphoproliferative disorder,which is uncommon and constitutes around  two percent of  hematolymphoid malignancies. HCL commonly involves bone marrow and spleen and  rarely peripheral blood . Splenomegaly is  a prominent feature and  is seen in around 70 to 100% of  HCL cases as reported  in various case reports. Sometimes the absence of splenomegaly rules out the diagnosis of HCL and   is misdiagnosed as aplastic anemia. Thus the aim of our study is to understand the importance and keep a high level of suspicion in such cases. As in present case there was no evidence of splenomegaly clinically or radiology, but the morphologic features on biopsy had suggested HCL which was further confirmed on immunophenotyping. The purpose of this case report is to highlight the importance of the fact that HCL can present even without splenomegaly. Keywords: Hairy cell leukemia, Splenomegaly, Immunohistochemistry


2021 ◽  
Vol 2 (3) ◽  
Author(s):  
Chase H. Foster ◽  
Anthony J. Vargas ◽  
Elizabeth Wells ◽  
Robert F. Foster ◽  
Suresh N. Magge

BACKGROUND The ability of coronavirus disease 2019 (COVID-19) to cause neurological insults in afflicted adults is becoming increasingly understood by way of an ever-growing amount of international data. By contrast, the pandemic illness’s neurological effects in the pediatric population are both poorly understood and sparsely reported. OBSERVATIONS In this case, the authors reported their experience with a preschool-age child with hydrocephalus who suffered multiterritory strokes presumed secondary to immune-mediated cerebral vasculopathy as a result of asymptomatic COVID-19 infection. LESSONS Growing evidence indicates that COVID-19 can cause neurological sequelae such as encephalitis and strokes. In this case report, the authors discussed a case of cerebral vasculopathy and strokes in a pediatric patient who was positive for COVID-19.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4383-4383 ◽  
Author(s):  
Zeina G El Amil ◽  
James N Butera

Abstract Abstract 4383 Introduction: Black cohosh (known as both Actaea racemosa and Cimicifuga racemosa), a member of the buttercup family is a perrenial plant that is native of North America. It has been widely used to relieve premenstrual problems, menstrual cramps and to control hot flushes and other menopausal symptoms. The mechanism of action of the black cohosh is not fully understood. Here, we report the case of a patient with pancytopenia related to the use of the black cohosh. Case Report: A 58 year old female patient with past medical history of dyslipidemia, presented with bruising after several months of taking black cohosh. Her complete blood count revealed a white blood cell count of 3.1×109/L (normal 3.5–11 ×109/l), hemoglobin of 10.6 g/dl (normal 11–15 g/dl), hematocrit of 31 % (normal 32–45%), mean corpuscular volume of 109 fL (normal 80–98 fl), platelets count of 37 000/μL (normal 150000–400000/μL). Her medications included lipitor, black cohosh and vitamin D. Her physical exam was remarkable only for bruising. Serum protein electrophoresis did not show any evidence of a monoclonal spike and a coomb’s test was negative. She had normal immunoglobulins level, haptogloin, LDH, liver function tests, iron studies, vitamin B12 and folate. Her prothrombin and activated partial thromboplastin times were within reference range. Her peripheral smear showed macrocytic red blood cells and decreased platelets. Her bone marrow biopsy and aspirate showed a hypocellular bone marrow (cellularity of 25–30%), with a predominance of erythroid precursors that exhibit shift toward immaturity and mild to moderate megaloblastoid changes, moderately decreased myeloid precursors, slightly decreased megakaryocytes, and normal iron stores, there was no increase in the bone marrow blasts (0.6%). She also had a flow sent for paroxysmal nocturnal hemoglobinuria, which showed normal levels of expression of CD55 and CD59 of the neutrophils and the red cells. Upon presentation, the black cohosh was stopped. Following this, her white count, hemoglobin and platelets improved slowly (figures 1,2,3,) without any further interventions, but it took over 2 years for her counts to normalize. Conclusion: Black cohosh is an over the counter herbal medicine that has been widely used by women to control premenstrual symptoms, menstrual cramping and menopausal symptoms. Many side effects have been described in the past but its effect on the bone marrow has never been studied. In our patient, Black cohosh caused pancytopenia that was reversible; however the recovery was very slow. Disclosures: No relevant conflicts of interest to declare.


2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Vimal Master Sankar Raj ◽  
Diana Warnecke ◽  
Julia Roberts ◽  
Sarah Elhadi

Goodpasture’s syndrome (GPS) remains a very rare disease entity in the pediatric population characterized by the presence of pulmonary hemorrhage and rapidly evolving glomerulonephritis. We hereby describe the case of a 2-year-old girl who presented with renal failure and was diagnosed with GPS. A brief review of the literature in regard to data on demographics, pathogenesis, clinical features, diagnosis, treatment, and prognosis for renal recovery is also provided.


2016 ◽  
Vol 2 (1) ◽  
pp. 57-59
Author(s):  
Pavithra D ◽  
Praveen D ◽  
Vijey Aanandhi M

Agranulocytosis is also known to be granulopenia, causing neutropenia in circulating blood streams .The destruction of white blood cells takes place which leads to increase in the infection rate in an individual where immune system of the individual is suppressed. The symptoms includes fever, sore throat, mouth ulcers. These are commonly seen as adverse effects of a particular drug and are prescribed for the common diagnostic test for regular monitoring of complete blood count in an admitted patient. Drug-induced agranulocytosis remains a serious adverse event due to occurrence of severe sepsis with deep infection leading to pneumonia, septicaemia, and septic shock in two/third of the patient. Antibiotics seem to be the major causative weapon for this disorder. Certain drugs mainly anti-thyroid drugs, ticlopidine hydrochloride, spironolactone, clozapine, antileptic drugs (clozapine), non-steroidal anti-inflammatory agents, dipyrone are the potential causes. Bone marrow insufficiency followed by destruction or limited proliferative bone marrow destruction takes place. Chemotherapy is rarely seen as a causative agent for this disorder. Genetic manipulation may also include as one of the reason. Agranulocytosis can be recovered within two weeks but the mortality and morbidity rate during the acute phase seems to be high, appropriate adjuvant treatment with broad-spectrum antibiotics are prerequisites for the management of complicated neutropenia. Drugs that are treated for this are expected to change as a resistant drug to the patient. The pathogenesis of agranulocytosis is not yet known. A comprehensive literature search has been carried out in PubMed, Google Scholar and articles pertaining to drug-induced agranulocytosis were selected for review.


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