White Blood Cell disorders, lymph nodes, spleen, thymus Flashcards
How does hematopoiesis occur? What is a normal WBC count?
- Stepwise maturation of CD34+ hematopoietic stem cells
- Cells mature and are released from bone marrow into blood;
Normal WBC count usually 5-10 K/uL
A. Low would be leukopenia
B. High would be leukocytosis
Low or high WBC count usually due to decrease or increase in one particular cell lineage
Give two examples of leukopenia; what is this caused by and possible treatment?
- Neutropenia (drug toxicity due to damage to stem cells leading to decreased WBC production caused by maybe alkylating agents, especially neutrophils; severe infection with increased movement of neutrophils into tissues resulting in decreased circulating neutrophils caused by gram-neg sepsis;
could treat with GM_CSF or G-CSF to boost granulocyte production) - Lymphopenia (immunodeficiency like DiGeorge or HIV; high cortisol state inducing lymphocyte apoptosis like with Cushing or exogenous corticosteroids; autoimmune destruction like with SLE; whole body radiation leading to lymphopenia
Neutropenia is like attempting to knock out Al-Qaeda elite members to prevent the number of ready-made Jihadists they trot out; also if fewer cops are off the street to fight crime, where are the others on the street?
Jeff George thought it would be cool to try out CORTICOIDS to help with his football. Instead, in DC, his body BROKE DOWN and the suckiness RADIATED throughout his whole body
Define neutrophilic leukocytosis and include some causes
Increased circulating neutrophils:
- Bacterial infection or tissue necrosis (induced release of marginated pool and bone marrow neutrophils, including mmatures forms (a left shift) and immature cells are characterized by DECREASED Fc receptors (CD16)
- High cortisol state (impairs LEUKOCYTE adhesion, leading to release of marginated pool of neutrophils)
For monocytosis, eosinophilia, basophilia, and lymphocytic leukocytosis, list the causes
Monocytosis: increased circulating monocytes (chronic inflamm states like autoimmune and infectious, and malignancy)
Eosinophilia: increased circulating eosinophils (allergic reactions like type I hypersens, parasitic infections, and Hodgkin lymphoma; think INCREASED EOSINOPHIL CHEMOTACTIC FACTOR);
Basophila: increased circulating basophils, seen in CML;
LL: increased circulating lymphocytes (viral infections with T lymphocytes undergoing hyperplasia, and bordetella pertussis infection with bacteria making lymphocytosis-promoting factor, which blocks circulating lymphocytes from leaving the blood to enter the lymph node)
CIMbad always stands alone in his ship (monocytosis);
I’m allergic to Hodgkin, that little parasite (Eosinophilia);
Lymphocytic leukocytosis: Don’t go into a Mexican bordella, you’ll get a virus!!
In infectious mono, what is the main cause? A less common cause?
EBV INFECTION results in lymphocytic leukocytosis with reactive CD8+ T cells; CMV a less common cause;
EBV transmitted by saliva, classically affecting teens
For IM, what does EBV primarily infect?
- oropharynx, resulting in pharyngitis;
- liver, resulting in hepatitis with hepatomeg and elevated liver enzymes;
- B cells
For IM, the CD8 T cell response leads to:
- generalized lymphadenopathy due to T-cell hyperplasia in lymph node paracortex
- splenomeg due to T-cell hyperplasia in periarterial lymphatic sheath (PALS)
- High WBC count with atypical lymphocytes (reactive CD8+ T cells) in the blood
What test is used to screen for IM?
Monospot:
- Detects IgM antibodies to cross-react with horse or sheep RBC’s
- Turns positive within 1 week after infection
- A negative monospot test suggests CMV as possible cause of IM
- Definitive diagnosis made by SEROLOGIC TESTING for EBV viral capsid antigen
Elisa B Varane has dated 8 guys in France and she’s KISSED all of them and SWAPPED SPIT. She’s had Blowjobs, known to DRINK ALCOHOL (liver) and has DIFFICULTY SPEAKING. Regarding the guys she’s dated, she’s still PALS with one of them, one of them was a good LAD, and the last was very WHITE and also a little AWKWARD. She’s now started hooking up with a guy from INTER MILAN, but even after just ONE WEEK he’s gone with her sister CECILIA M Varane. What’s left? Test for the EBV viral capsid antigen.
Complications of IM
- Increased risk for splenic rupture (patients should avoid contact sports for one month)
- Rash if exposed to AMPICILLIN
- Dormancy of virus in B cells leads to increased risk for both RECURRENCE and B-cell lymphoma, especially if immunodeficiency devleops
Raphael Varane needs to not play for A MONTH or he’ll RUPTURE his SPLEEN. If he’s too amped, he’ll get a RASH. If he screws too many ladies, he might have something wrong with his BALLS.
What does acute leukemia consist of? What is this subdivided into?
neoplastic proliferation of blasts, defined as accumulation of >20% blasts in the bone marrow;
increased blasts crowd out normal hematopoiesis, leading to anemia, thrombocytopenia, or neutropenia (fatigue, bleeding, infection);
blasts enter blood stream, leading to high WBC count (blasts are large, immature cells, often with punched out nucleoli);
ALL or AML based on blast phenotype
On a CROWDED beach, it’s easy to get TAN; you’ll be surrounded by LARGE, IMMATURE guys who tend to PUNCH each other.
In ALL, what accumulates and what are these cells characterized by? When does ALL usually arise?
Neoplastic accumulation of lymphoblasts (>20%) in the bone marrow with positive nuclear staining for TdT, a DNA polymerase; TdT absent in myeloid blasts and mature lymphocytes;
children and associated with Down syndrome
How is ALL subclassified? Describe the two differences?
B-ALL and T-ALL
1. B-ALL: you see lymphoblasts with TdT, CD10, CD19, and CD20; has an excellent response to chemo and requires prophylaxis to scrotum and CSF;
prognosis is better with t(12;21) which is more common in kids compared to t(9;22), more commonly seen in adults
2. T-ALL: lymphoblasts with markers ranging from CD2 to CD8, and blasts not expressing CD10; usually seen in teenagers as a mediastinal (thymic) mass, or ACUTE LYMPHOBLASTIC LYMPHOMA!!
ALL Tyshawn Taylor wants to do is provide for his SIX YEAR OLD kid with DOWN SYNDROME. Thus, he loves to BALL with Elliot Williams and Riley Cooper (20 and 19). The kid will be okay once he’s 12-21, but he won’t be good if they move to Philadelphia.
For AML, what does this consist of and what are the cells characterized by? Who do we see this in?
Neoplastic accumulation of immature myeloid cells (>20%) in bone marrow; characterized by positive cytoplasmic staining for myeloperoxidase (crystal aggregates of MPO could be seen as AUER rods!!);
look at older adults (50-60 years)
How is AML subclassified?
- APL: the t(15;17) translocation of RAR on chromosome 17 moving to 15, and promyelocytes can accumulate;
the abnormal promyelocytes have numerous primary granules that increase risk for DIC; treatment is ATRA, a vit A derivative that can bind altered receptor and cause blasts to mature!!) - Acute monocytic leukemia: prolif of monoblasts USUALLY LACKING MPO, and the blasts typically infiltrate GUMS!!
- Acute megakaryoblastic leukemia: proliferation of megakaryoblasts and lack MPO, associated with Down syndrome
- AML could also arise from pre-exiting dysplasa (myelodysplastic syndromes), especially with prior exposure to alkylating agents or radiotherapy
The problem with Mason Plumlee is he’s IMMATURE, but he did get a CAlL by RED AUERbach, who’s 55!! Red said that on the SURFACE Plumlee is good, but he just needs to grow a bigger DIC!! Otherwise, he’ll stay SINGLE with GUM in his mouth(free agent) and remain an AWKWARD big man. At least he won’t be MICAH DOWNS.
MDs can be a problem for Mason: they’re ABNORMAL, HYPER, SHALLOW, and BLAST you for everything you do wrong.
With myelodysplastic syndromes, what is the presentation and how do patients die?
- cytopenias, hypercellular bone marrow, abnormal mautration of cells, and increased blasts (<20%)
- infection or bleeding leads to death
In chronic leukemia, what does this consist of, what is it characterized by, and who do we see it in?
Neoplastic proliferation of mature circulating lymphocytes;
high WBC count;
usually insidious in onset, seen in older adults!!
For CLL, what is happening and what is seen? Complications?
Neoplastic proliferation of naive B cells co-expressing CD5 and CD20 (most common leukemia);
Blood smear: increased lymphocytes and smudge cells;
involvement of lymph nodes leads to generalized LAD and is called small lymphocytic lymphoma;
Comps: hypogammaglobulinemia (infection most common cause of death in CLL), autoimmune hemolytic anemia, and transofmration to diffuse large B-cell lymphoma (a Richter transformation) with enlarging lymph node or spleen
Mason Plumlee (5) and Andre Dawkins (20) were hanging out with Jeremy Maclin (19) when MJ came around (23), who is the most COMMON jersey seller. Dawkins wanted to SMUDGE Jordan’s reputation and called him SMALL!! During 1 on 1, Dawkins, normally AUTOMATIC from three, HURT HIMSELF and GOT an infection. Meanwhile, Mark RICHT was pissed Jeremy Maclin didn’t commit to Georgia.
For hairy cell leukemia, what is happening and charaterized by what? Clinical features and treatment?
Neoplastic prolif of mature B cells with hairy cytoplasmic processes!!!;
cells positive for tartrate-resisitant acid phosphatase;
clinical features: splenomeg because of hairy cell accumulation in red pulp and “dry tap” on bone marrow aspiration (marrow fibrosis); LAD USUALLY ABSENT;
Treat: 2-CDA (cladribine) an adenosine deaminase inhibitor and adenosine accumulates to toxic levels in neoplastic B cells!!!!!
The trouble with Hairy is he’s MATURE enough to go to SPain, but he feels TRAPped there where the air is also DRY. But he just doesn’t have time to Live his LYFE. His cure? Give him scantily CLAD (2-CDA, or cladribine) so adenosine can accumualte.
For adult TLL, what’s happening and assocations and clinical features?
Neoplastic proliferation of mature CD4+ T cells;
HTLF-1 association, and seen in Japan and Caribbean most commonly;
clinic: rash (skin infiltration), generalized LAD with HSM, and lytic (punched-out) bone lesions with hypercalcemia
When I was 4, I felt mature enough to move from Atlanta to Japan and the Caribbean. My mom thought that was RASH, and was tempted to PUNCH me, but I ended up going on HMS with my dad the GENERAL.
For mycosis fungoides, what’s going on and how does the patient present?
Neoplastic prolif of mature CD4+ T cells that infiltrate the skin, producing localized skin rash, plaques, and nodules;
aggregates of neoplastic cells in epiderm are PAUTRIER MICROABSCESSES;
cells can spread to involve blood, meaning Sezary syndrome (lymphocytes with cerebriform nuclei, or Sezary cells, seen on blood smear)
Chuck Thompson IV infiltrated/hacked PSN and ended with a pautry haul of money. It would have even pissed off Julius Sezar (Sezary syndrome with cerebriform nuclei in lymphocytes)
What’s the basis for myeloproliferative disorders? Complications?
Neoplastic prolif of mature cells of MYELOID lineage, a disease of late adulthood (average age of 50-60 years);
HIGH WBC COUNT with hypercellular bone marrow (cells of all myeloid lineages increased and classified based on the dominant myeloid cell made);
increased risk for hyperuricemia and gout due to high turnover of cells, and progerssion to marrow fibrosis or transformation to acute leukemia
50-60 yrs old, Kareem Abdul-Jabbar is pretty MATURE and he’s getting WHITER. Hope he’ll still be able to walk and support his wife CRYSTAL, or he’ll become STIFF and transform into something worse
CML is what? What are some presentations? How is it different from leukemoid reactions?
Neoplastic prolif of mature myeloid cells, especially GRANULOCYTES AND THEIR PRECUROSRS; basophils characteristically increased!!
See splenomeg, and the spleen getting larger suggests progression to acclerated phase of disease, and transformation to acute leukemia usually follows after (can transform to AML in 2/3, ALL in 1/3);
distinguished by negative leukocyte alkaline phosphatase, increased basophils, and t (9;22)
CML: doesn’t get a LAP dance, plays the BASs, and is a TRANS (9;22)
Polycythemia vera is what? What is it associated with? Clinical symptoms? Treatment? Distinguish it from reactive polycythemia how?
Neoplastic prolif of mature myeloid cells, especially RBCs (granulocytes and platelets also go up);
the JAK2 kinase mutation;
consider 1. blurry vision and headache 2. increased risk of venous thrombosis 3. flushed face due to congestion (plethora) 4. itching, especially after bathing due to histamine release;
treat with phlebotomy, second-line is hydroxyurea (otherwise, death within a year);
distinguish by the EPO levels decreased and O2 sats normal, with reactive polycythemia due to high altitude or lung disease having opposite levels and also ectopic EPO production from renal cell carcinomoma with O2 sats normal and EPO high
Randy Big Couture is JAKED; if you get TOO MUCH OF HIM, you’re not going to be able to SEE STRAIGHT, have HEADACHES, you won’t have BLOOD GOING to the right places, you’re going to be FLUSH in the face from the PLETHORA of punches he throws, and when you take a bath for the soreness you’ll be ITCHING. GET RID OF HIM (phlebotomy) and maybe HYDROXYUREA. Otherwise, you’re dead within the YEAR!!
