Darius
The structure of the red blood cell, reminiscent of a pizza crust
biconcave disc
Two most commonly encountered types of mutations seen in JAK2
V617F, Exon 12
This translocation, when present in gastric MALT lymphoma, predicts a low response rate to H Pylori eradication therapy
11;18
the molecular weight, in kiloDaltons, often measured of the fusion protein that results from this translocation, when it is found in the setting of acute lymphoblastic leukemia, rather than in myeloid malignancy
190 kD BCRABL
A potential drug target in AML, this protein is named after a fictional character whose silhouette resembles the protein's spiked structure
Sonic Hedgehog Protein
the action by which macrophages consume
phagocytosis
The immunophenotype of CLL
CD5+, CD20dim+, CD23+, CD200+, light chain restricted
Two lymphomas against which pembrolizumab has a high efficacy
Primary mediastinal B cell lymphoma, Classical Hodgkin lymphoma
this therapy has a distinct advantage over eculizumab in that it can be given less frequently
ravulizumab
The absence of these on electron microscopy, along with pulmonary fibrosis and one other classic exam finding, would make one suspect Hermansky Pudlak syndrome. (name the EM finding and the exam finding)
dense grannules on platelet electron microscopy, occculocutaneous albinism
Refers to the stick shaped inclusion bodies seen in acute myeloid leukemia, which inspired an unfortunate name for these cells which thankfully is no longer used
Auer rods
The mechanism of action of teclistamab
anti BCMA bite
This disorder presents classically with concurrent bone marrow failure and pancreatic insufficiency
Schwachmann Diamond syndrome
This rare disorder is caused by mutation in ferrochelatase
Erythropoetic protoporphyria
In allogeneic stem cell transplantation, Permissive mismatches in this HLA subgroup are associated with improved clinical outcomes
HLA-DPB1
the nutritional deficiency associated with megaloblastic anemia
B12 deficiency
Two treatments that are used during acute attacks in porphyria patients
dextrose infusion
Elevation of this cytokine is a diagnostic criteria of POEMS syndrome
Serum VEGF
These criteria comprise the diagnosis of "definite" catastrophic antiphospholipid syndrome
•Involvement of ≥3 organs, systems, or tissues
•Manifestations develop simultaneously or over <1 week
•Small vessel occlusion is confirmed histologically in at least one organ or tissue
•Presence of aPL (anticardiolipin antibodies, anti-beta2-glycoprotein I antibodies, and/or lupus anticoagulant) is documented twice, at least 12 weeks apart
Of these five genetic mutations, all of which cause hereditary iron overloading syndrome, only this one is associated with high hepcidin levels (name all 5)
HFE
HJV (hemojuvelin)
HAMP (hepcidin)
TFR2 (transferrin receptor 2)
SCL40A1 (ferroportin, high hepcidin)
The target of rituximab
CD20
This 2nd generation CML therapy is classically known to be associated with risk of pleural effusions
dasatinib
This mutation is implicated in congenital neutropenia syndromes including cyclical neutropenia syndrome
ELANE
The lack of S-100 staining is a key in distinguishing this rare histiocytic disorder from its counterpart, whereas the presence of this mutation is commonly found in both of these disorders, both of which are named after people (name the mutation and the disorder)
Erdheim Chester Disease, BRAF mutation
In fetal hematopoesis, during mid to late gestation, hematopoesis occurs primarily here
Liver