Fire Facts Flashcards by ricardo rangel

What protein allows RBCs to change shape as they pass through vessels?

Spectrin

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What name is given to immature erythrocytes in circulation?

Reticulocytes

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What pathologic form of RBC would you see in the following diseases? Lead poisoning

Basophilic Stippling

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What pathologic form of RBC would you see in the following diseases? G6PD Deficiency

Bite cells and Heinz Bodies

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What pathologic form of RBC would you see in the following diseases? DIC or TTPHUS

Schistocytes - fragmented RBC

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What pathologic form of RBC would you see in the following diseases? Abetalipoproteinemia or liver disease

Acanthocytes (spur cells) - irregularly spiked

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What pathologic form of RBC would you see in the following diseases? Asplenia

Howell-Jolly bodies and Target cells

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What pathologic form of RBC would you see in the following diseases? Uremia/Renal Failure

Echinocytes (burr cells) - regular uniform spikes

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What pathologic form of RBC would you see in the following diseases? Rounded cell that has lost its concave shape

Sherocytosis - Hereditary Spherocytosis

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What pathologic form of RBC would you see in the following diseases? Liver disease

Schistocytes or acanthocytes

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What pathologic form of RBC would you see in the following diseases? Infarcted spleen

Howell-Jolly bodies

Splenectomy is Tx for: Immune thrombocytic purpura (ITP), Sickle cell anemia, or Px with spleen trauma

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Px presents with anaphylaxis on exposure to blood products with IgA.
What pathology is at hand?

Selective IgA deficiency

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Px presents with corse facial features, abscesses, eczema.

What pathology is at hand?

Hyper-IgE Syndrome (Job Syndrome)

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Px presents with thrombocytopenia, purpura, infections, eczema.
What pathology is at hand?

Wiskott-Aldrich syndrome

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Px presents with delayed separation of the umbilicus.

What pathology is at hand?

Leukocyte adhesion deficiency type I.

Phagocyte dysfunction disorder.

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Px presents with Neuro defects, partial albinism, recurrent infections.
What pathology is at hand?

Chédiak-Higashi Syndrome

Also a phagocyte dysfunction disorder

What 3 hematologic changes would you expect to see in a Px without a functional spleen?

Thrombocytosis
Howell-Jolly bodies
Target cells

Where does fetal erythropoiesis take place??

“Young liver synthesizes blood”

Yolk Sac (3 -8 weeks)
Liver and Spleen (migrated to liver from mesonephros after a couple of weeks)
Bone marrow (~28wks)
Vertebrae, ribs, and pelvis (axial skeleton in adults)

PT describes the following symptoms:
Encephalopathy, memory loss, headaches, foot/wrist drop, abdominal colic, and renal colic (eventually has renal failure). What pathology is at hand?

Lead poisoning
These symptoms are the non-hematologic signs which also include:
Lead lines on bone X-ray, and on gingivae (Burton lines)

What triggers sickling of RBCs?

Hypoxemia
Dehydration
Acidosis

May cause a sickle cell crisis

Pt presents with cyanosis of the fingers and toes, with hemolytic anemia. What disease is this?

Cold autoimmune hemolytic anemia

—> cold involves IgM
—> Warm involves IgG

Pt presents with hematuria in the mornings, and fragile RBCs. What disease is at hand? What is happening?

Paroxysmal nocturnal hemoglobinuria

RBC are undergoing premature hemolysis by way of complement, and thus thrombosis, impaired bone marrow function, and hemolytic anemia make the disease life threatening.

Basophilic nuclear remnants in RBCs. What is this called?

Howell-Jolly bodies

Pt undergoes autosplenectomy… what is the associated disease? What is the treatment of choice?

Sickle cell

Tx of choice for crisis: hydroxyurea

Pt presents with lower extremity purpura, arthralgias, and renal disease, what is the most likely diagnosis?

Henoch-Schonlein purpura (type of vasculitis)

What are the 3 constitutional B symptoms?

Low grade fever Night sweats Weight loss

What is the translocation in Philadelphia chromosome? What is the specific mutation? What disease is more commonly seen with this mutation?

t (9;22) —> creates a mutation in bcr-abl (an oncogene) to be always active Seen in: CML (philly CreaML cheese)

Why is AML more common in Pts with Down syndrome?

The translocation at t (8;21) would give 3 copies of the translocation, thus making AML more common in these patients

Under microscopy, plasma cells show a nucleus that is packed with a “clock face” appearance of chromatin in the nucleus. The cytoplasm just outside the nucleus is cleared out and is called the peri nuclear hof. RBC have a Rouleaux formation (stacked like coins). Lyric bone lesions like Swiss cheese on xrays, like the skull. What is the pathology at hand?

Multiple myeloma

What are the 4 MC clinical features of Multiple myeloma? What urine test is helpful for diagnosis?

-Anemia -Renal insufficiency -Back pain (due to incr. osteoclast func, dec. OB function -Hypercalcemia (Also, monoclonal antibody spike “M-Spike” on serum protein electrophoresis Immunoglobulin light chain in urine (no proteinuria), but you see Bence-Jones proteins by way of urine protein electrophoresis (UPEP) Seen In an older Px usually

How do you differentiate Multiple Myeloma from Waldenström Macroglobulinemia?

Waldenström macroglobulinemia does not have lytic bone lesions, and has proliferation of IgM. Also associated with amyloidosis and hyperviscosity.

A solid tumor of plasma cells may occur by 2 ways... name them.

Plasmacytoma - Solitary plasmacytoma of bone - Extramedullary plasmacytoma (predilection for the head and neck - nose) —> do not cause lytic bone lesions

Pathology shows: Monoclonal proliferation of plasma cells Production of monoclonal immunoglobulins No symptoms of multiple myeloma and no end organ damage. can progress to multiple myeloma. What is the pathology at hand?

Monoclonal Gammopathy of Undetermined Significance (MGUS)

What is the translocation of AML-M3 (Acute Promyelocytic AML)? What is the treatment?

t (15;17) | Tx: All-trans retinoic acid

Patient presents with increased WBC count (30-50k) as a result of a stressful event. This can also be a cause of infection, Down Syndrome, Malignancy, Kawasaki Disease, heat stroke, or congenital abnormalities T of Fallot). These cells are predominantly neutrophils and labs show a 5-10% band shift. What is the most likely diagnosis??

Leukemoid Reaction

What is the clinical consequence of protein C or protein S deficiency?

Hypercoagulable state